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SYPHILIS

Brief Historical Outline

There are three theories regarding the origin of syphilis. Their essence consists in the following.
According to one concept, syphilis was brought to Europe by Christopher Columbus's sailors in 1493 after his
discovery of America. The seamen were allegedly infected by the aborigines who, since they were prone to
bestiality, contracted the disease from llamas (it has long been known and proved that llamas have spirochaet-osis).
In confirmation of this theory, its advocates, who were promptly dubbed 'Americanists', pointed to the circumstance
that after Columbus's expedition returned home many cases of syphilis were registered in the port towns of Spain.
The disease was then spread to Europe by the mercenary soldiers of Charles VIII, King of France who laid siege to
Naples after conquering Rome. According to the testimony of contemporaries, the hired soldiers revelled orgies in
Rome, where 14 000 prostitutes had been brought from Spain. The king was forced to lift the siege because of the
"terrible disease" that decimated his army. The disbanded soldiers brought the dis-ease to many other European
countries, causing an epidemic, according to some data, even a pandemic. Thus, this theory contends the homeland
of syphilis is Central America (the island of Haiti).
The second theory advanced in the sixties asserts that syphilis originated in Africa. These authors contend that
the causative agents of tropical or endemic treponematoses (framboesia, pinta, and that of venereal syphilis are
variants of the formerly identical treponema organism. In their opinion, the beginning of treponema parasitism in
humans reaches back to the early Neolithic period, in the later evolution of treponematosis is closely connected with
the evolution of human society. Thus, when people first settled inareas with a dry and cooler climate,
treponematosis was manifestin the form of bejel, and with the growth of towns and cities it developed into venereal
syphilis.
Africa is still the most intensive focus of endemic treponematoses. Hudson claims that Africa is their
homeland. The 'Africanists' hold the view that the spread of treponematoses to Europe and to the Asian countries
through Arabia was facilitated by the migration of people from Africa. Syphilis spread as a consequence of wars,
crusades, trade links, slave-traders siezing black slaves and taking them to the American and Asian countries, and the
pilgrimage of Christians to the shrines of Jerusalem and of moslems to Mecca. It is advisable to take close notice of
the morphological and biological identity of T. pallidum which causes venereal syphilis and the treponema
organisms causing tropical treponematoses transmitted mainly by the non-sexual route, of the similarity of the
clinical and pathomorphological pictures in syphilis and framboesia, bejel, pinta, of the positive serological blood
tests, including the T. pallidum immobilization tests in all treponematoses, and of the efficacy of drug therapy for all
treponematoses with arsenic preparations in the past and antibiotics now. This identity suggests that rom the
standpoint of the evolution of microorganisms the origin of these treponema organisms is probably the same and
their 'separation' could have taken place in times of very long ago due to various conditions of existence.
Finally, the third theory states that syphilis allegedly existed since prehistorical times in the countries located
on the territory of Europe, Asia, and the Middle East. Many scientists share this view (they are called the 'Europists')
and their arguments are very convincing. One cannot ignore the statements made by such emi nent scholars of ancient
times as Hippocrates, Gales, Celsus, Susruta, Dioscorides, Plutarch, Archigenes, and Avicenna who described their
works diseases (ulcer, aphtha, condyloma and others) bearing a close resemblance to syphilitic diseases.
Avicenna, for instance, described lesions that are very much like hard chancre, condyloma latum, papular syphilids,
and gumma. Descriptions have been preserved of lesions of the bones remindful of syphilitic affections of the bone
system. There is evidence that syphilis was known in East India and that it was treated with mercury long before the
discovery of America (ten centuries . C.). Manifestations very similar to syphilis are mentioned in Chinese
manuscripts relating to 2600 . . and in a book on Japanese medicine (808 B.C.).
In vessels discovered during excavations conducted in Central Asia, Borzov found mixtures with a 30 per cent
mercury content. This provided grounds for assuming that mercury preparations had been used for treating syphilis
in ancient times. The "Europists" possess very valuable proof of their hypothesis in the remains f skeletons in which
changes very typical of syphilis were revealed by X-ray. During archaeological excavations in the area byond
Lake Baikal, Soviet scientists D. G. Rohklon and A. E. Rubasheva found a bone with gummatous periostitis. This
was the tibia of a man who lived sometime about 1500 . . In studying remains found during excavation of burial
grounds relating to the I century . . in the Verkhneudinsky District, archeologists disclosed tibiae with gummatous
ostitis and periostitis, and in another instance a frontal bone with multiple syphilitic destructive changes.
Thus, none of the stated theories has been given universal recog-nition despite the proofs backing them. In all
probability syphilis appeared on Earth at about the same time that man did, and the different theories of its origin
simply provide historical information.
The term 'syphilis' was introduced by Fracastorius, an Italian physician, poet and astronomer, who wrote a
poem in 1530 in Verona about a shepherd named Syphilus whom the gods punished with a disease of the sex organs
for his impertinence to them.
Until that time, syphilis had been called the Spanish, Italian, French (Gallic), Portuguese or some other nation's
disease. Since the name 'syphilis' did not offend any national feelings, this is evidently why it survived the test of
time.
There were certain periods in the history of syphilis, which great-ly influenced the research on the problem as a
whole. In Fracastorius' time and nearly three centuries after his death, physicians and scien-tists disputed the
question whether syphilis was to be regarded an independent disease or a particular manifestation of a general
disease comprising syphilis, gonorrhoea and soft chancre. Adherents of the first viewpoint were called dualists and
of the second - Unitarians. In an attempt to end this controversy, Hunter, a Scottish scientifist of great prestige in
1767 introduced the pus from a gonorrhoea pa-tient into his own urethra. A few days later a discharge appeared and
in a few weeks hard chancre developed. We realize today that an unfortunate error occurred: the material had been
taken from a patient who had probably been suffering simultanusly both from gonorrhoea and syphilis. At that time,
however (the causative agents of gonorrhoea, syphilis and soft chancre had not yet been discovered, the methods of
serological diagnosis were still not de-vised, etc.), this self-sacrificial experiment carried out by a well - known
scientist created such an impression that it weighed the scales in favour of the Unitarians and thus delayed the
correct solution of the problem. The term 'lues' was first introduced in reference to syphilis by Rabelais but became
commonly accepted in medical usage after Fernel used it in his book in 1554.
It was only 60-70 years later that Hunter's experiment was given a correct interpretation. This became possible
as a result of the re-search conducted by French physician Ricord who in the period from 1831 to 1837 infected 700
people with syphilis and 667 with gonorrhoea. The dispute between the Unitarians and the dualists was finally
settled. Scientists could not ignore the results that were obtained by Ricord, but censured the impermissible method
that had been used to establish the truth.
Besides Hunter, the young French physician Lindeman made a notable contribution to the history of
syphilology. In 1851 he in-fected himself with syphilis under the control of a committee ap-pointed by the French
Academy (placing the discharge taken from syphilitic papules into a cut made in his hand). Student Mezenov also
infected himself with syphilis to test the prophylactic effect of mercury subchloride ointment suggested by I. I.
Mechnikov. The second half of the 19th and the beginning of the 20th centu-ries were characterized by the intensive
study of venereal diseases, including syphilis, and the discovery of the causative agents of in-fectious diseases. The
works of Fournier should be noted; he made a complete and accurate clinical description of all the periods of syphilis
and its different manifestations, which is of great value to this day. The works of Russian scientists V. M.
Gratsiansky, P. M. Shiryaev and V. P. Fedorov are also of great merit. In 1875 V. M. Gratsiansky was the first in
world medical literature to de-scribe the changes in the afterbirth if the mother had syphilis. P. M. Shiryaev (1881)
and V. P. Fedorov (1903) investigated this problem in detail. In 1903 I. I. Mechnikov and French scientist Roux
obtained positive results in inoculating two chimpanzees with syphilis, and in 1904 D. K. Zabolotny achieved the
same in a baboon; this opened the way to the study of experimental syphilis in animals closely related to man.
The discovery of the causative agents of gonorrhoea (1879) and oft chancre (1885-1887) made it possible to
reject the unitarian theory without question.
One of the great discoveries in the history of syphilology was made in March, 1905 by Schaudinn and
Hoffmann who found treponema, me causative agent of syphilis; they called it spirochaeta pallidum for its inability
to stain well with different dyes. The question of the aetiology of syphilis was thus resolved, making it possible to
carry on research into the clinical and diagnostic aspects of this infection on a new foundation.
A major event became the discovery by Wassermann in collabo-ration with Neisser and Brooke (1906) of the
serological reaction to syphilis, which became known as the Wassermann reaction, or test. This also Jed to the
discovery of many other specific serologjcal reactions. A qualitatively new test for syphilis was the Treponema
pallidum immobilization test proposed in 1949 by the Americans Nelson and Mayer. It was conducted first in the
USSR in 1959 by A. I. Kartamyshev and G. B. Belenky. This test proved to be far more specific than the ordinary
'classic' serological reactions. It is of major significance for the recognition of pseudopositive non-syphilitic
serological reactions.
As regards the late forms of syphilis, note should be made of the first description made in 1822 by Bayle of
progressive general paralysis, the setting apart by Duchenne de Boulogne in 1858-1859 of tabes dorsalis as an
independent nosological form, and the discovery of Noguchi in 1913 of Treponema pallidum, in the cerebral
parenchyma in patients suffering from general paresis and tabes dorsalis.
An important advance in studying syphilis of the nervous system was the examination of cerebrospinal fluid in
patients with various forms of syphilis. The fluid was collected by means of lumbar punc-ture (spinal tap). The
importance of this method of examination carried on since 1891 (Quincke) has grown particularly after studying
Wassermann's reaction in the cerebrospinal fluid (1906) and Lange's colloidal test (1912).
There were two outstanding discoveries in the treatment of syph-ilis, which deserve special notice. The
German researcher and physician Ehrlich inaugurated a new era in the therapy of syphilis by proposing
arsphenamine in 1909 and neoarsphenamine in 1912. Although treatment of syphilis with organic compounds of
penta-tomic arsenic has been discontinued everywhere, it should be remembered that arsenic together with mercury
and bismuth prepa-rations helped successfully to treat patients with various forms of syphilis for more than four
decades.
The use of penicillin (which was discovered by Englishman Fleming in 1929) by American physicians
Magoney, Arnold and Harris in 1943 became a new method in principle in the treatment of syph-ilis. In the Soviet
Union Z. V. Ermolyeva and T. P. Bazelina (1942) are to be merited for obtaining penicillin. For the first time ever
the mould Penicillium glaucum was applied with a therapeutic purpose in 1872 by Polotebnov. Antibiotics currently
take the lead in the treatment of syphilitic patients. The assortment of therapeutic means has become very extensive.
An important role is played by long-acting penicillin preparations (ecmonovocillin, bicillins). Soviet bicillin was
synthesized first in 1954 by I. S. Ioffe and F. K. Sukhomlinov (Leningrad) and then in 1955 by Z. V. Ermolyeva and
E. N. Lazareva (Moscow).

Development of Syphilology in the USSR1


The scant available information on the early history of syphilis in Russia generally comes down to the
admission that the disease was brought from Western Europe. The first written mention of the appearance of syphilis
in Russia dates back to 1490. Most reearchers usually refer to M. N. Karamzin, the well-known Russian historian,
who, in describing the reign of Tsar Ivan III in Volume VI of the 'History of the State of Russia' (in Russian)
mentioned that according to a Lithuanian historian, in 1943 'a woman brought the French disease from Rome to
Krakow. This horrendous disease visited many persons, among them Cardinal Frederick too... . It is fairly possible
that this is why, among the numerous assignments given by Ivan III to his ambassador to Lithuania boyar Mamonov,
there was the order to make sure when stopping at Vyazma on his way to Lithuania to find out 'whether anyone had
come with the disease that is called French and allegedly brought from Vilna'.
The 'sexual plague' penetrated Russia rapidly because it was precisely in these years that trade began
intensively developing with the Western countries. 'The boundaries of Russia are open for free trade of all nations by
land and by sea. We play host to the merchants of sultans and caesars, to Spanish, German, French, Li-thuanian,
Persian, Bukhara, Khiva, Shemakhan and many other traders, so we can get along without the English and shall not
close the gates into our country to others just to please the English', Tsar Fedor wrote to Queen Elizabeth of England
who demanded that English merchants be granted exclusive rights to trade with Russia. It was in this period that
syphilis in Russia assumed the character of a national calamity.
Indirect indications of syphilis in Russia were found in decrees concerning apothecaries issued from 1645 to
1674 which referred to certain specialists ('healers') who treated syphilis by administering quinine tincture and
decoctions per os.
By the middle of the 18th century the incidence of syphilis in Russia attained threatening proportions; this can
be judged by the that, according to a decree of the senate dated May 20, 1763,omen sick with 'france-veneria'
engaged in prostitution were, on being cured, to be exiled to Nerchinsk. The materials serving as that for this
decision of the senate resulted from an investigation had been made of the St. Petersburg general hospital which led
that "of the 671 in patients at this establishment more than two thirds had contracted france-veneria from wanton
women, as sertified by the head physician...". The first medical book about syphilis, the appearance of which was
acclaimed as 'diligence for public good and for the encouragement of others', came out in Russia in 1755. It was
published (in Russian), by Venechansky under the title 'Methods and Instructions by means of which Peasants and
Others Suffering from the French Disease Can Be Treated by Their Landlords and Stewards and can keep away from
this disease'.
The second half of the 19th century is regarded as the time when scientific venereology began developing in
Russia. The first department of venereal diseases was organized at the Medico-Surgical Academy in 1869 and Prof.
V. M. Tarnovsky was elected to head it. V. M. Tarnovsky is the founder of the Russian school of syphi-lology, a
gifted scholar and clinician, a skilled researcher and an outstanding organizer of venereal disease prevention. He is
the author of the first Russian textbooks on venereal diseases and of nu-merous works devoted to syphilis and
gonorrhoea. V. M. Tarnovsky's activities were versatile. 'There is not a single chapter in syphilolo-gy that had not
been thoroughly researched by Tarnovsky himself, or his pupils', stated A. I. Pospelov at a sitting of the Moscow
Der-matological Society. Tarnovsky initiated the convocation of the First Congress in 1897 for working out measures
of syphilis control. The congress discussed the routes and causes of the spread of syphilis mainly among the rural
population, where not only a high in-cidence had been registered, but a malignant course of infection with
predominance of late crippling forms of syphilis. In 1885 the first Scientific Society of Dermatovenereologists in
Russia was organized in St. Petersburg. After V. M. Tarnovsky, T. P. Pavlov headed the department of skin and
venereal diseases of the Military Medical Academy and continued research in the general pathology of syphilis.
A substantial contribution to the development of venereology was made by the departments of dermatology
and venereology that were organized in Kiev (1865), Moscow (1869) and other cities. The Moscow school of
venereologists was headed by Prof. A. I. Pospelov. Among the well-known Russian venereologists were M. I. Stuko-
venkov (Kiev) in whose clinic methods for treating syphilis with mercuric preparations were studied; I. F. Zelenev
(Kharkov), publisher of the first dermatovenereological journal (1901) in Russia, who studied the effect of arsenic
preparations in the treatment of syphilis; A. N. Ge (Kazan) who wrote the manual 'A Course in Venereal Diseases' (in
Russian); 0. V. Petersen (St. Petersburg) who discovered (1887) the causative agents of soft chancre, namely, the
Petersen-Ducrey streptobacillus, and suggested bismuth powders for external treatment of syphilids; M. S.
Rozenblum (Odessa), who was the first to apply pyretotherapy for treating patients with neurosyphilis; D. K.
Zabolotny, M. A. Chlenov and P. S. Grigo-ryev, who worked in the field of experimental syphilis. It was only after
the Great October Socialist Revolution, however, that planned and coordinated work began in the prevention of
venereal diseases. This work is now being carried on by dermatological and ve-nereological institutes under the
Central Institute of Skin and Ve-nereal Diseases, the departments of skin and venereal diseases at medical higher
schools and institutes of advanced medical training, as well as by a large army of dermatologists and venereologists
staffing the republication, regional, town and district dermatological and venereological out-patient clinics and other
medical establish-ments for skin and venereal diseases in the USSR.

THE AETIOLOGY OF SYPHILIS


March 3, 1905 is the date when the causative agent of syphilis, the Treponema pallidum (syn. Spirochaeta
pallidum), was discovered. It is a spiral thread shaped like a corkscrew when viewed under an optical microscope
(Fig. 29). On the average, it has from 8 to 14 uniform spirals each about l m long. This determines the length of the
treponema, which is about 7 to 9 um long and 0.2 to 0.5 mm thick, Great motility is characteristic of T. palltdum.
Several types of its movements are distinguished, namely, back and forth (sometimes this is a thrust-like, swinging
movement); pendulum-like, bending, contractile (wave-like, spasmodic) and around its own axis (rotary). The
smoothness of movements distinguishes the T. pallidum from other saprophytic spirochaeta (Spirocheta dentium,
buccalis, refringens, balanitidis), which are characterized by coarser morphological features, non-uniform size of the
spirals and the absence of smoothness in movements.
In the external environment outside the body T. pallidum dies rapidly. Drying kills it. Heating to 60C destroys
it within 15 minutes and at 100C instantly. Various disinfectants (0.5 per cent phenol solution, corrosive sublimate
and 1:4000 mercuric chloride solutions, 66 per cent ethyl alcohol, potash soap suds, 0.3-0.5 per cent solution of
various acids, etc.) produce an equally rapid effect. T. pallidum is more resistant to low temperatures. For instance, it
remains pathogenic after being stored at 78C for a year. In a moist discharge, treponema lives for up to 12 hours
and longer. Animals may be infected from a cadaver within the first 48 hours.
Electron microscopy which magnifies T. pallidum 25 000 to 30 000 times allowed Ovchinnikov and
Delektorsky to study the structure of the causative agent of syphilis in detail (Fig. 30). It was found that it is covered
with a triple-layered coat. The layers differ in composition and function. Fibrils are found under the layers, next
comes the cytoplasmic membrane covering the cytoplasm. Fibrils are threads by which the treponema moves and
attachess it-self to blepharoblasts present in the distal parts. It is believed that the blepharoblasts are not only the sites
of fibril fixation, but serve as centres of movement at the same time. The cytoplasmic membrane like the outer coat
is also composed of three layers. Ribosomes (gran-ules of various size) responsible for the synthesis of protein mole-
cules, the nuclear vacuole and mesosomes are clearly distinguished in the composition of the cytoplasm. The
mesosomes are important structures performing a complex function. They originate from the cytoplasmic membrane
and are analogues of the mitochondria of the body cells and perform the function of respiratory, metabolic, and other
functions by means of enzymatic systems localized in them. Mesosomes participate in the multiplication of
treponema and spor-ulation together with the cytoplasmic membrane.
Treponemas multiply by transverse division. Besides the principle type of division, other ways of
multiplication are possible as well as the sexual process (Ovchinnikov). Under the influence of unfavourable
conditions of existence (the effect of antibiotics, under-nourishment, etc.), treponemas may produce 'survival forms',
i.e. cysts and L-forms, Cysts are formed when the treponema organisms gather in a coil (this process was studied by
electron microscopy) and are covered by a common mucin-like capsule which is imper-meable to drugs. As cysts,
treponemas may exist for a length period of time evidently without causing a marked pathogenic effect on the body.
There is likewise no body reaction to the organisms. A tempo-rary conditional 'balance' between the micro- and
macro-organism occurs. If the body's defence forces are weakened, however, or if unfavourable factors no longer act
on the microorganisms, pathogenic treponemas may again form from the cysts, i.e. reversion occurs. Therefore, the
cysts are not degenerative forms but forms of the stable survival of the treponemas (Ovchinnikov). Hence the need to
give patients with syphilis (particularly during the first therapeutic courses) adequately high doses of antibiotics
because small concentrations are conducive to the occurrence of treponemas sensitive to the given drug and cause
their transformation to cysts and L-forms.
Ovchinnikov's and Delektorsky's experimental findings are in agreement with the works of authors who
believe that syphilis may follow a long-term asymptomatic course (if L-forms of T. pallidum are present in the body)
and that the disease may be accidentally detected in the latent stage (lues latens seropositiva, lues ignorata), i.e. in
the period when treponemas exist in the body probably as cyst forms possessing antigenic properties and,
consequently, lead-ing to the production of antibodies. This is confirmed by positive serological tests for syphilis in
the blood of patients with no notice-able clinical manifestations of the disease. Moreover, stages of neurosyphilis and
viscerosyphilis are revealed in some patients, i.e. the disease develops as if 'by-passing' the active forms (for a
detailed discussion of the subject see section on general course of syphylis).
Complex conditions (special media, anaerobic conditions, etc.) are essential for obtaining T. pallidum cultures.
At the same time, cultured treponemas rapidly lose their morphological and pathogen-ic properties. The existence
of granular and invisible filterable forms of T. pallidum, besides the forms mentioned above, was presumed.

Laboratory Identification of Treponema Pallidum


Active lesions of syphilis are examined for T. pallidum (which is mainly found in a primary syphiloma, eroded
papules and con-dilomata lata) for verifying the diagnosis of the early forms of syph-ilis (primary and secondary
stages). Staining methods have lost their practical significance because these organisms stain poorly with aniline
dyes (hence, the designation 'pallidum'). Moreover, an important differential sign of these treponemas allowing them
to be distinguished from vulgar spirochaetes, namely, a diverse mo-bility is lost on staining. T. pallidum is examined
in all laborato-ries in the living state, with an ordinary light microscope in dark field illumination produced by means
of a paraboloid condenser or a disk of thick black (photographic) paper (Arkhangelsky's method). With the use of the
paraboloid condenser and side illumi-nation by a lamp a variety of shining hard particles (leucocytes, epithelial cells,
tiny particles) and T. pallidum are detected in the dark field in a drop of serous exudate collected from the focus.
The surface of erosions, ulcers, eroded papules, etc., found on the skin or mucous membranes is wiped with
cotton or gauze tampons moistened in isotonic sodium chloride solution. This manipulation is repeated several times
because it is profoundly important in the iden-tification of the treponemas. Not only are the scales, pus, and san-
guineous crusts removed from the focus by this procedure, but also the saprophytic spirochaetes lying on the surface;
T. pallidum in contrast is an intratissue parasite. The surface examined is then carefully stroked with a platinum loop
or spatula till a serous fluid appears. A drop of this fluid is placed on a glass slide and examined for . pallidum. In a
negative result the examination is repeated several times. Before repeating the examination a dressing moistened in
an isotonic sodium chloride solution is applied for 24 hours to the surface of the lesion for its better cleansing. A
negative result of the examination for T. pallidum does not rule out the diagnosis of syphilis, because the patient may
have used various ointments and disinfectant solutions for self-treatment.
In such cases the diagnosis of the disease is made from examina-tion of the aspirate of a regional lymph node, the
clinical picture, the results of blood tests for syphilis, the findings of the examina-tion of the partner in sexual intercourse
(confrontation), etc.

CONDITIONS AND ROUTES OF INFECTION WITH SYPHILIS


T. pallidum enters the human body through injured skin or mucous membranes. The infection atrium may be so
negligible that it remains unnoticed by the examiner. A syphilitic patient is contagious, particularly in the period of active
manifestations, when treponemas are 'washed out' together with the serum from the deeper parts of the tissues onto the
surface of the lesions as the result of rubbing (in walking), friction (during the sex act), irritation (mechanical or
chemical); the treponemas may also be spread in food (when syphilitic papules occur in the oral cavity).
Sexual contact of a syphilitic patient with a healthy person should now be recognized as the main route of infection.
Gases of infection through objects (commonly shared dishware, cigarettes, smoking pipes, etc.) are rare, which is
explained by the improved sanitary education of the population and the considerably upgraded sanitary and hygienic
conditions of life. (In prerevolutionary Russia, this mode of infection prevailed, particularly in villages). Infection may be
transmitted by kisses if the patient has eroded syphilitic lesions in the mouth. Cases when treponemas found in the
secretions of syphilitic lesions contaminate objects of everyday use, which thus convey infection, are comparatively rarer.
Treponemas remain viable for a lengthy period of time in a moist medium outside the human body. Physicians and other
medical personnel may contract the disease during examination of a syphilitic patient or during therapeutic manipulations.
Such instances have occurred among midwives, surgeons, obstetricians and gynaecologists, stomatolo-gists and
occasionally among venereologists and laboratory assistants handling material containing T. pallidum. Such infection is
avoided if extreme care is taken not to injure the skin of the hands and if the hands are wiped with a 1:1000 mercuric
chloride solution and thoroughly washed with soap after examining a patient (particularly one with a contagious stage of
syphilis).
Cases of infection with syphilis in direct transfusion of blood of a donor suffering from syphilis are extremely rare.
It is considered that the saliva of a patient is contagious because of the presence of treponemas in it only if there are
syphilitic lesions in the mouth. It has been suggested that the milk of a nursing mother suffering from syphilis is
contagious even when there are no noticeable syphilitic lesions in the region of the nipple.
A similar interpretation is given to the contagiousness of the semen despite the absence of manifestations of the
disease on the gen-itals of a patient with active syphilis. At the same time, it is beIieved that the urine and sweat of
patients are not contagious.
It has been established that the amount of the causative agent introduced into the body of an experimental animal is
of importance in the development of the syphilitic infection. It may be assumed that this is also of definite importance in
humans. That is why the possibility of acquiring syphilis is much higher in persons who had repeated sexual intercourse
with a patient suffering from the active form of syphilis, than in those who had single or brief sexual liaison. At the same
time, the absence of criteria of infection with sy-philis obliges venereologists to subject to preventive treatment all
individuals who had had sexual intercourse with a patient suffering from an active form of syphilis as well as individuals
(children in particular) who had contact of any other kind.

EXPERIMENTAL SYPHILIS
Attempts to inoculate different animals with syphilis for produc-ing an experimental model of infection (for
studying its aetiology, pathogenesis, treatment, etc.) had been made as early as the last century. The first experiments to
win universal recognition, how-ever, were conducted by Mechnikov and Roux in 1903. The authors not only succeeded in
infecting two chimpanzees, but observed the development in them of the manifestations of the secondary stage of the
disease (papules on the abdomen and lower limbs). Later, Zabolotny (1904) engaged in the field of experimental syphilis
and Mechnikov (1905) infected baboons and Macacus monkeys (lower monkeys) with syphilis. The manifestations of the
disease were less pronounced in them than in anthropoid apes.
The possibility of experimental infection of monkeys with syphilis was finally confirmed after the discovery of T.
pallidum and the establishment of serological diagnosis of syphilis. As a result valuable information was obtained on
various problems of syphilology (contagiousness of the internal organs, involvement of the nervous system, prophylactic
significance of mercurous chloride ointment, active immunization in syphilis).
Experimental syphilis began to be utilized most widely only after the discovery of a method for infecting rabbits
with syphilis. This possibility was proved in 1906 by Bertarelli who infected the animals with infectious material
introduced into the cornea and anterior chamber of the eye. Parodi is merited for producing primary syphiloma and
regional adenitis in rabbits after injection of the infectious material into the testis. Rabbits are now the animals principally
used in the experimental study of syphilitic infection and testing of antisyphilitic drugs. Although they are very helpful in
interpreting the problems of pathogenesis and treatment of syphilis the results of rabbit experiments should be
applied with caution in relation to humans.

GENERAL COURSE OF SYPHILIS


The body's response to the entry and multiplication of T. pallidum is characterized, as a rule, by the change of
active clinicallly manifested periods of the disease to periods in which there are no manifestations on the skin and
visible mucous membranes (latent periods). The pronounced character of the symptoms and the severity of the
affections grow with time and not only their clinical, but their pathoanatomical essence alters, which is associated
with immunological body reactivity. The French syphilologist Ricord drew attention to the regular change of periods
in the 'classical' course of syphilis According to this classification by periods, the incubation, primary secondary and
tertiary periods (stages) of syphilis are distinguished.

The incubation period is length of time between infections and formation of the primary syphiloma (hard
chancre) and lasts 4 to 5 weeks on the average. Primary syphilis lasts 6 to 8 weeks, from the moment that primary
syphiloma develops until the appearance of skin and mucosal eruptions typical of the secondary period of syphilis. If
no treatment is applied, the secondary periods developsdevelops 6 to 8 weeks after the appearance of the hard
chancre. The significance of serological tests in the diagnosis of syphiliswas pointed out above. Wassermann's
reaction and serological precipitin reactions are negative in the first two or three weeks after appearance of the hard
chancre (primary, seronegative syphilis seu lues I seronegativa), but become positive later (primary seropositive, seu
lues I seropositiva). At first the blood serological tests for syphilis are weakly pronounced. Gradually their titre
grows to such an extent that by the end of the primary and the beginning of the secondary periods they yield a
sharply positive result (4+) in almost 100 per cent of cases.
Besides the primary syphiloma, enlargement of lymph nodes (bubo) closest (regional) to the site of the entry of
the infection is characteristic of the active period of syphilis. Regional scleradenitis (usually in the inguinofemoral
and less frequently in the submandibular region) develops mostly one week after the formation of the primary
syphiloma. As the result of generalization of the infection and the corresponding immunological reaction of the body,
various lymph nodes become enlarged (polyadenitis) at the end of the primary period and systemic symptoms (a
temperature reaction, headache, pain in the muscles and joints, weakness, indisposition, rapid fati-guability) appear
often. These general symptoms of syphilitic infection in the period of its generalization are often interpreted as
influenza and with the eruption of rose coloured spots or papules the secondary period is sometimes erroneously
diagnosed as measles, scarlet fever, rubella, toxicoderma and even as enteric fever or typhus.
The primary active period of syphilis may be followed by a latent period (syphilis latens), though most
freguently the disease develops directly into secondary syphilis (syphilis secundaria, seu lues I This period is
characterized by the appearance of diffuse and gener-alized eruption on the skin and mucous membranes usually of
rose coloured spots (roseola) and papules, much less frequently of pustular lesions. Secondary syphilis, in which
eruptions appear on the skin and mucous membranes for the first time, is called the secondary early period (syphilis
secundaria recens). In about 2 or 3 months the eruption disappears spontaneously (without treatment) and the disease
develops into the secondary latent period (syphilis seu lues II latens). Recurrence of eruptions in secondary syphilis
is known as secondary recurrent syphilis (syphilis II recidiva seu lues II recidiva)
The duration of the latent period in secondary syphilis varies due to complex immunobiological processes and
the interrelationship between the macro-organism and T. pallidum. It may last several months, or even until
secondary syphilis develops into tertiary syphilis. In untreated cases the secondary period of syphilis lasts two to
three years, on the average, sometimes five years. Recurrent eruptions may occur during this period (secondary recurrent
syphi-lis) if antisyphilitic treatment is not applied. Three, four and more such recurrences may develop. The lesions of
secondary recurrent syphilis (mostly rose coloured spots and papules) differ from those of secondary early syphilis in a
lesser number, larger size, paler colour, tendency to form groups, figures, arches and garlands, and localization restricted
to places of intensified irritation (anogenital region, oral mucosa). There are other distinguishing symptoms, which are
discussed in the respective section of the textbook.
The tertiary period of syphilis (syphilis III, seu lues tertiaria) usually occurs no earlier than three to five years after
infection. Even if no treatment is applied, however, the disease may remain latent for many years and after a lengthy
period (at times over the span of several decades) it becomes manifest by tubercular or gum-matous lesions. These are
localized not only on the skin or mucous membranes, but in the internal organs, nervous system, bones and joints.
Syphilitic affections of the nervous system and internal organs may develop in some patients in the absence of tertiary
active eruptions. Cases have been reported, however, in which manifestations of tertiary syphilis or clinical symptoms of
involvement of the visceral organs and nervous system were not detected although antisyphilitic treatment had not been
given.
The diversity of the clinical course of a syphilitic infection (intervals between recurrences, the character of the
morphological lesions in different periods of active syphilis, the time of onset and character of tertiary syphilis, and much
besides) is not only determined by the immunological state of the body, the state of cell reactivity and the relationship of
the body with the causative agent of the infection in particular, but also by the external and internal factors. Among these
are: the patient's age, working and living conditions, various diseases (acute and chronic, infectious in partic ular),
toxicosis (especially alcoholism) and various types of trauma (psychic, physical), which are capable of aggravating the
cause of syphilitic infection.
The practicing venereologist occasionally encounters cases in which treponemas enter directly into the blood. This
usually occurs i transfusion of blood taken from a donor suffering from syphilis This condition is called transfusion
syphilis. It is characterized by the development of syphilitic infection without the formation of primary syphiloma or
regional scleradenitis. In eight to ten weeks eruptions characteristic of secondary syphilis form on the skin and mucous
membranes of the patient; the eruptions are preceded prodromal phenomena (elevated body temperature, headache, pain
in the bones and joints).
Malignant syphilis is now a rare occurrence. It is characterized by a shorter primary period (three to four weeks),
prevalence of pustular lesions, serve general symptoms, high fever, absence of polyadenitis or very weak signs of lymph
node involvement and, in some cases, negative results of serological blood tests. Diminished reactivity of the patient's
body and exhaustion or weakening of its immunobiological resistance under the effect of various detri-mental influences
(systemic diseases, infections, toxicosis, etc.) are believed to play a role in the pathogenesis of a malignant course of
syphilis.
Milich has been contending since 1968 that syphilis can develop without the regular replacement of one period by
another.

He claims that after infection the disease follows a protracted asymptomatic course in some patients and is
diagnosed much later on the basis of positive serological blood tests (in the stage of latent sero-positive syphilis which, in
this case, is called syphilis innocentum, or lues ignorata because neither the patient nor the physician knows when the
infection had occurred) or in the stage of syphilis of the nervous system or the viscera. Milich assembled data from the
litera-ture in addition to his own observations to the effect that most Patients (70-90 per cent) currently suffering from
late forms of syph-ilis lack a history of early syphilis, whereas, according to Fournier's data, a century ago 60 to 80 per
cent of patients with late syphilis had a history of early forms. The author thinks that it that the patients were unaware that
they had had early stages of syphilis, but that they had actually not suffered from them Moreover he draws attention
to a large group of patients identified on the grounds of positive serological blood tests whose marital partner does
not contract the disease despite long-time sexual contact and whose children are healthy, as a rule. (The author
argues: the manifestations of syphilis had been unnoticed by the patient how come the partner's body had also been
ignorant of the disease and failed to contract it) The probability of such an assumption is now also built on
experimental data which demonstrate that syphilis in rabbits may follow an asymptomatic course.

Immunity in Syphilis. Superinfection. Reinfection


A person cured of syphilis does not acquire immunity; congenital immunity is also absent. Repeated infection
(reinfection) is possible, and this is proof that syphilis is curable. Infection with syphilis for a second, and even for a
third time, has been described.
At the same time immunity develops in the patient's body in the period of syphilitic infection; this immunity is
called non-sterile, infectious. It is a reaction of the body to the presence of the causative agent of the disease; it exists
as long as the causative agent remains in the body and disappears as soon as the patient recovers. Infectious
immunity is attended with an allergic reaction. Infectious allergy vanishes with the disappearance of infectious
immunity.
In the incubation period of syphilis the treponemas which had entered the body spread rapidly by the
lymphogenous-haemato-genous route. The body's reaction in the form of primary syphiloma and regional
scleradenitis is delayed. Mass multiplication of treponemas and their spread throughout the body, i.e. a peculiar
spirochaetal sepsis, occur at the end of the primary and the beginning of the secondary period of syphilis. This causes
the development of general symptoms of the disease (temperature reaction, weakness, indisposition, pain in the
bones and joints, etc.) and polyadenitis in this period. The presence of the pathogenic agent (T. pallidum) leads to the
mobilization of immunobiological defence mechanisms in the patient's body, as a result of which most of the
treponemas die and the latent period of secondary syphilis sets in. The surviving treponemas, however, become
adapted to the new conditions of existence and multiply with the gradual decrease in the intensity of the defence
processes in the macroorganism and cause a recurrence (secondary recurrent syphilis). After this, the immunobiolog-
ical forces of the macro-organism are again mobilized, and again, if no treatment is applied, treponemas (possibly
the cyst forms and others), which had remained in the body, contribute to the continued presence of the syphilitic
infection. The wave-like course of the infection in the secondary period of the disease reflects, there-
fore, the complex interrelationship between the micro- and the macro-organism. The character of affection in
the tertiary period of syphilis, when treponemas are detected in the macro-organism with great difficulty (they cannot
be detected in these patients, as a rule), is evidently explained by the fact that in tertiary syphilis the body is
considerably weakened and hypersensitized to the treponema and its toxins and reacts even to a small number of the
causative agents by a peculiar infectious granuloma (tubercle and gumma) characterized by disintegration, necrosis,
tissue damage, and subsequent cicatrization.
When the body of a syphilitic patient receives additional infection (a 'dose' of a new T. pallidum strain), as if a
new syphilitic infection is superimposed on the old one, a condition called superinfection develops. The
manifestation of superinfection differs with different periods of the disease. It is believed that in superinfection the
body reacts to the entry of new treponemas by eruptions of that stage, that period, which the patient is undergoing at
the time (e.g. if superinfection occurs in the period of secondary latent syphilis, morphological lesions of the
secondary period, i.e. papules, rose-coloured spots, etc. appear). Thus, in the incubation period and in the first ten to
fourteen days of the primary period of syphilis, when there is still no pronounced infectious immunity, repeated
infection leads to the successive development of a new chancre which is smaller and usually develops after a shorter
(up to 10-15 days) incubation period. Such chancres are called successive (ulcera indurata succentuaria),
Superinfection may also be manifested in the late tertiary period when the few foci of infection (even gummatous)
are incapable of maintaining the body's immunobiological reactivity at a high level.
The practicing venereologist encounters cases in which it is extremely difficult to differentiate superinfection
with a recurrence of the disease from reinfection. This is explained by the absence of absolute ly reliable tests and
criteria proving that syphilis has been cured.
The following premises are essential in making the diagnosis of reinfection:
(1) the early form of syphilis is diagnosed with the first infection, namely, primary, secondary active (with
identification of T. pallidum) or early latent syphilis;
(2) adequate quality treatment is applied for the early form of syphilis;
(3) transformation to negative serological reactions and disappearance of the syphilids should take place
within the usual limit of time (no resistance to specific therapy is encountered);
(4) the classical serological tests (Wassermann's reaction with 1 antigens and the precipitin reactions) must be
stably negative in the period of observation over the patient. After treatment is completed, the T. pallidum
immobilization test (TPI) and the imunofluorescence test (IFT) also become negative.
(5) no less than a year should elapse after treatment for the first-infection has been completed;
(6) in a second infection it is necessary to disclose the new source of infection with active form of syphilis and
identify T. pallidum
(7) the length of time from sexual intercourse with a person suff ering from a contagious form of syphilis to the
moment when reinfection is diagnosed should correspond to the usual terms of the diagnosis of primary
seronegative, primary seropositive, secondary fresh secondary recurrent or early latent syphilis;
(8) if primary syphiloma develops in reinfection, it has a different localization than that in the first infection (it
may form at some distance from the primary localization, but not in the same place). The development of the hard
chancre in such cases is attended with regional scleradenitis.

CLASSIFICATION OF SYPHILIS
A standard classification exists in the USSR for registration and record of patients with syphilis. The following
forms are distinguished after the incubation period.
1. Primary, seronegative syphilissyphilis I seronegativa.
2. Primary, seropositive syphilissyphilis I seropositiva.
3. Primary latent syphilissyphilis I latens. This diagnosis is made when the treatment is begun in the
primary period of the disease in the absence of subsequent clinical manifestations of syphilis.
4. Secondary fresh syphilissyphilis II recens.
5. Secondary recurrent syphilissyphilis II recidiva.
6. Secondary latent syphilissyphilis II latens. It is diagnosed in patients whose treatment was begun in the
secondary fresh or recurrent period in the absence of clinical manifestations of syphilis at the given time.
7. Tertiary active syphilissyphilis III active.
8. Tertiary latent syphilissyphilis III latens. This diagnosis is made in patients who have no clinical
manifestations of the disease but had revealed active manifestations of the tertiary period in the past.
9. Latent syphilissyphilis latens:
(a) Early latent syphilissyphilis latens praecox;
(b) Late latent syphilissyphilis latens tarda.
This diagnosis is established in cases with no clinical manifestations of the disease, but with positive
serological tests.
10.Early congenital syphilissyphilis congenita praecox: congenital syphilis of infants (under 1 year of age)
and very young
11. Late congenital syphilissyphilis congenita tarda.
12. Later congenital syphilissyphilis congenita latens.
13' Visceral syphilis (with indication of the involved organ).
14. Syphilis of the nervous system.
15. Tabes dorsalis.
16. General paresisparalysis progressive.

PRIMARY PERIOD OF SYPHILIS (SYPHILIS PRIMARIA)


The primary period of syphilis begins from the moment that the primary affect (hard chancre) forms at the site
of entry of T. pallidum and lasts until the symptoms of the secondary period appear. According to the results of blood
test for Wassermann's reaction and precipitin reaction, seronegative and seropositive periods are distinguished in it.
In syphilis following a classical course, the duration of the primary period is 6 to 8 weeks, on the average. It is
preceded by an incubation period which is considered to be 4 to 5 weeks, on the average. In individual patients
weakened by tuberculosis, rheumatism, alcoholism or hypovitaminosis, the incubation period may be shortened to
less than three weeks.
A prolonged incubation period in syphilis of over 4 to 5 weeks, however, is recorded more frequently.
Medication with antibiotics (orally or by injection) for tonsillitis, colds, pneumonia, etc. is particularly conducive to
an increase of the incubation period. Such cases are encountered most frequently in simultaneous infection with
syphilis and gonorrhoea the incubation period of which lasts just a few days. The doses of antibiotics which these
patients receive are sufficiently effective in gonorrhoea but cannot eliminate the syphilitic infection in the patient's
body even in the period of incubation, but simply lead to prolongation of this period (sometimes to several months).
Some authors observed prolongation of the incubation period to 52-120 days in individuals who did not take
antibiotics for intercurrent diseases. Preventive treatment is currently applied in cases in which two to four months
have passed from the moment of sexual intercourse with a patient suffering from a contagious form of syphilis as
well as in patients with gonorrhoea when the source of infection is not known and the patient for some reason cannot
be kept under surveillance for a lengthy Period of time.
The active primary period of syphilis is manifested clinically by the primary syphiloma (hard chancre),
regional scleradenitis and, sometimes, by lymphangitis developing from the hard chancre to the adjoining enlarged
lymph nodes.

Hard Chancre (Ulcus Durum), or Primary Syphiloma


After the incubation period, the primary syphiloma (hard chancre) forms at the site of entry of T. pallidum into
the skin or mucouos membranes. The hard chancre is usually localized on the skin and mucous membranes of the
genitals (glans penis, preputial sac, the anus in homosexuals, labia majora and minora, posterior commissure, cervix
uteri), less frequently on the thighs, pubis or abdomen. The extragenital chancre, which is a less frequent occurrence,
forms on the lips, tongue, tonsils, eyelids, fingers and on any other area of the skin or mucous membranes which the
treponemas had penetrat-ed. This is referred to as extragenital localization of the primary syphiloma. In such cases as
well as in localization on the neck of the uterus (found in 11 to 12 per cent of cases, according to the data of some
authors) the primary syphiloma remains unnoticed, which some authors claim to occur in 95 per cent of female
patients. The stated above also explains, to some degree, the rather high level of latent syphilis revealed not only in
examination of sex contacts when early latent syphilis is predominantly diagnosed, but also in prophylactic
examination of definite strata of the population, in performing Wassermann's reaction in somatic departments of
hospitals where late latent or syphilis innocentum is prevalently diagnosed. For instance, according to some authors,
the incidence of 'serological' syphilis has increased 5.6 times among males and 2.9 times among females within the
last nine years.
The clinical picture of hard chancre is very characteristic, as a rule. It is usually manifested by single, strictly
round or oval (Plate IV), saucer-like erosion with discrete boundaries, and the size of the little finger nail. The
erosion has the colour of raw meat or spoiling fat, its edges are slightly elevated and sloping towards the floor
(saucer-shaped). It produces serous sparse secretions which lend the chancre a shiny ('polished') appearance. The
most characteristic sign of the hard chancre is an infiltrate of denseelastic consistency palpated in the base of the
erosion (hence the name 'hard' chancre, primary 'sclerosis'). The edges of an ulcerous hard chancre are elevated even
more, while the infiltrate is more pronounced. On healing an ulcerous hard chancre leaves a scar while an erosive
chancre heals without a trace. The course of the primary syphiloma is characterized by mild tenderness or total
absence of subjective disturbances. T. pallidum is easily detected in secretions of the primary syphiloma in dark-field
illumination.
The clinical picture of hard chancre has considerably changed over the years. According to many authors, one
of the substantia features of primary syphiloma was that it was always single (in 80-90 per cent of cases), whereas in
the last decade, in contrast, the number of patients with two or more chancres has markedly increased (34 per cent
according to Skripkin and Glozman, and to 45.7 per cent of cases according to Rakhmanov and Zudin). A
considerable increase in the specific share of ulcerous chancres and their complica tion by pyogenic infection is also
observed. The number of patients with chancre of the anogenital region has grown. A definite number of chancres of
oral and anal localization is associated with sex perversions. The specific share of chancres in the mouth, for
instance, is considerably higher among females. The anus is the most frequent localization of an extragenital chancre
in males. The absence, in some cases, of an obvious induration in the base of a primary syphiloma (in 5 per cent of
patients, according to Skripkin) is among the peculiarities of the course of primary syphilis today. Atypical forms of
primary syphiloma are comparatively rare.

Atypical Chancres
Several variants of atypical chancre exist: chancre-amygdalitis, chancre-panaritium and indurative swelling.
Chancre-amygdalitis is characterized by enlargement and hardening of one tonsil with no erosion or ulcer on it
(if an erosion or ulcer of the primary period of syphilis is found on the tonsil, this is called primary syphiloma
localized on the tonsil). No marked inflammation around the tonsil, temperature reaction or painful swallowing are
in evidence, while the tonsil is sharply demarcated. Its resilience is felt on palpation with a spatula. A large number
of treponemas are easily found on the surface of the tonsil in such cases after it has been lightly stroked with a
platinum loop. The presence of regional scleradenitis on the neck at the mandibular angle, typical of the primary
period of syphilis (lymph nodes ranging in size from a large bean to a hazel nut, mobile, of dense, elastic
consistency, not fused with the surrounding tissues, painless), and positive serological blood tests help in making the
diagnosis.
The hard chancre may be found on the fingers in the usual clinical form, or in an atypical dorm (chancre
panaritium). This localization is mostly found in medical personnel (laboratory workers, gynaecologists,
stomatologists, etc.). In clinical picture chancre-panaritium resembles the common panaritium of streptococcal aetio-
logy (club-shaped swelling and sharp tenderness of the distal phalange), but the presence of a hard infiltrate, the
absence of an acute inflammatory erythema and, most important, the presence of characteristic regional (cubital
lymph nodes) scleradenitis make its recognition easier. Despite the differential signs, the diagnosis of chancre-
panaritium may be very difficult.
If it is suspected, the results of the Wassermann test should be borne in mind in making the diagnosis. The
recognition of such cases is sometimes delayed until eruptions of the secondary period of syphilis appear.
Indurative swelling as a manifestation of the primary period of syphilis is found in the region of labia majora,
scrotum or prepuce i.e. in places richly supplied with lymph vessels. These areas become swollen. Manifest
induration of the tissues with no pitting on their compression are characteristic. The characteristic regional sclera-
denitis, medical history, results of examination of the sex partner, and the positive results of serological blood test for
syphilis (in the second half of the primary period) also help in the diagnosis of atypical hard chancre manifested as
indurative swelling.
In some patients primary syphiloma is complicated by secondary bacterial infection. This condition is called
complicated hard chancre.

Complications of Hard Chancre


Balanitis, balanoposthitis, phimosis, paraphimosis, the development of gangrene, phagedena are complications
of hard chancre. Balanitis and balanoposthitis are the most commonly encountered complications. They develop as a
result of attendant coccal or trichomonadal infection. In such cases swelling, bright erythema, and maceration of the
epithelium develop around the chancre. The secretion on the surface of the chancre becomes seropurulent, which
makes detection of T. pallidum and, consequently, the diagnosis much more difficult. Lotions with isotonic sodium
chloride solution are applied for one or two days to relieve the inflammation, which in most cases, makes it possible
to establish the correct diagnosis in repeated tests.
Balanoposthitis may lead to constriction of the prepuce so that the foreskin cannot be retracted. This condition
is called phimosis (Fig. 33). The swelling of the prepuce in phimosis gives the appearance of an enlarged penis
which is red and painful. The hard chancre localized in such cases in the corona glandis or on the inner surface of the
prepuce cannot be examined for T. pallidum. The diagnosis of syphilis is made easier by the characteristic regional
lymph nodes whose aspirate is examined for the causative agent. The respective therapy may also be prescribed
(which delays the diagnosis of syphilis): sulphanilamide emulsion, warm baths with isotonic sodium chloride
solution, oral sulphanilamides. The phenomena of phimosis are relieved as a result and the primary syphiloma is
exposed and may be examined. An attempt to retract the prepuce in phimosis with force may lead to another
complication called paraphimosis, in which the oedematous and infiltrated preputial ring strangulates the glans. As
the result of mechanical disorders of blood and lymph circulation, the swelling increases. Necrosis of the tissues of
the glans penis and prepuce may occur if measures are not applied in time. In the initial stages of paraphimosis the
physician removes the serous fluid from the swollen prepuce (by puncturing the thin skin with a sterile needle
repeatedly) and attempts to 'reduce' the glans. If the manipulation proves ineffective, the prepuce must be cut.
The development of gangrene and phagedena are more severe but less frequent complications of hard chancre.
They are encountered in weakened patients and alcoholics as the result of attendant fu-sospirillary infection. A dirty-
black or black scab (gangrene) forms on the surface of the chancre and may spread beyond it (phagedena). The scab
covers an extensive ulcer and the process may be attended with elevated body temperature, chill, headache and other
general symptoms. A coarse scar remains after the gangrenous ulcer heals. Treatment consists in immediate
prescription of penicillin.

Regional Scleradenitis
Regional scleradenitis is the second most important symptom of primary syphilis. It develops 7 to 10 days after
the appearance of the hard chancre. Lymph nodes closest to the hard chancre (usually the inguinal nodes) enlarge to
the size of a bean or a hazel-nut and become denseelastic, but do not fuse with one another, the surrounding tissues
or skin. They are painless and the overlying skin is normal. Regional scleradenitis persists for a long time and
resolves slowly despite specific therapy. When the hard chancre is localized on the neck of the uterus and rectal
mucus, regional scleradenitis cannot be detected clinically because in this case the lymph nodes of the small pelvis
are enlarged. Ever since Ricord's time regional scleradenitis has been known as 'attendant bubo', a term which aptly
conveys the meaning. Ricord wrote that scleradenitis is a constant attendant of the chancre, shadowing it unfailingly
and that hard chancre does not exist without bubo. Fourniet noted the absence of regional scleradenitis only in 0.06
per cent of 5000 patients with primary active syphilis. According to some authors, however, during the last decades
regional scleradenitis has not been found in 1.3 to 8.0 per cent of patients with primary syphilis.
In localization of primary syphiloma on the genitals, inguinal scleradenitis is usually bilateral (even when the
hard chancre is located on one side). This occurs because the lymphatic system has well-developed anastomoses.
Unilateral scleradenitis is encoun-tered less frequently and is usually found on the side on which the chancre is
localized; only in exceptional cases is it of a 'cross' char-acter, i.e. found on the side contralateral to the chancre. The
num-ber of patients with unilateral scleradenitis has noticeably increased recently (according to Skripkin they
account for 27 per cent of patients with hard chancre).
The third symptom of the primary period of syphilis, syphilitic lymphangitis (inflammation of the lymphatic
vessels), develops as a hard, painless cord as thick as a bulbous-end probe. Small, bead-like thickenings sometimes
form along the length of the strand. In approximately 40 per cent of males lymphangitis is localized on the anterior
surface of the penis (in genital hard chancre).
The differential diagnosis of hard chancre in typical cases is easy and based on very characteristic
symptomatics. In an atypical clinical picture or complicated chancres, however, a differential diagnosis has to be
made with a number of diseases.
In herpes simplex of the genitals a group of vesicles appear on a pink spot. After their rupture, small grouped
erosions form; they may coalesce into a large erosion which has to be differentiated from primary erosive syphiloma.
Herpetic erosion is difierentiated from the latter by its microscalloped borders, strands of epidermis, inflammatory
hyperaemia around the erosions, the absence of induration or only mild induration of the base, pain, negative results
of examination for '. pallidum and the absence of regional scleradenitis. The examination for treponemas in such
cases, however, must be repeated several times (like the serological blood test for syphilis) so as not to miss herpetic
hard chancre, which forms in herpes simplex and resembles the erosion very closely in its clinical picture.
In scabies ecthyma (a scabies burrow complicated by secondary infection) on the glans penis or other parts of
the genitals differen-tial diagnosis has to be made with hard chancre. The presence of a purulent discharge, the
absence of induration at the base of the lesions, and the other symptoms of scabies (e.g. the patient's com-plaint of
generalized itching which intensifies at night) are taken into account.
Superficial ulcers on the labia minora and majora in acute Lipschuetz-Chapin's ulcer of the vulva occur in
young and teenage girls after a cold or because of poor hygienic habits. The eruptions are preceded by a chill,
indisposition, and elevated body temperature often to a high level. The edges of the ulcer are loose, eroded, the floor
is granular and covered with seropurulent secretions, the base is soft. An acute inflammatory reaction develops
around the ulcers. The lesions cause pain, particularly on palpation or in walk-ing. Detection of B. crassus makes it
possible to establish the cor-rect diagnosis.
Acute eresive (circinate) balanoposthitis develops in weakened individuals with poor hygienic habits. Erosions
with discrete polycyclic edges form. The acute character of the ulcerous lesions, ele-vated body temperature,
tenderness of the ulcers and regional lymph nodes, and the absence of T. pallidum in the secretions help in making
the diagnosis.
Chancriform pyoderma is rare, but presents the most difficulties in differential diagnosis with hard chancre.
The disease is of streptococcal aetiology. A single erosion or, most frequently, ulcer forms which is clinically very
similar to primary syphiloma. It has round contours, hard non-eroded edges and floor, scanty serous or seropu-rulent
secretions. Regional scleradenitis with all the features of syphilitic scleradenitis develops quite frequently. The
infiltrate always extends beyond the boundaries of the ulcer. The diagnosis is ultimately established on the basis of
multiple negative results of examination for T. pallidum, absence of treponemas in the as-pirate from the lymph
nodes, and negative results of repeated blood tests for Wassermann's reaction.
In some cases hard chancre has to be differentiated from cancerous or gummatous ulcers, particularly when
they are localized on the genitals, the lip or the nipple of a woman's breast. The ulcers in skin carcinoma are
characterized by hard edges and floor, mild inflammatory reaction in the surrounding tissue, tendency to bleed, slow
and torpid course, and involvement of lymph nodes in the process several months after the formation of the ulcer.
The edges and floor of a gummatous ulcer are also thickly infiltrated, though not as severely as in carcinoma. The
presence of the gummatous core the different character of ulcer formation in distinction from ulcer syphiloma (see
the section dealing with tertiary syphilis) and, finally, a different histological structure of the lesion help in estab-
lishing the correct diagnosis.
We do not deal here with the differential diagnosis of hard chancre with soft-chancre ulcers, because they had
practically been liquidated in the Soviet Union and may be encountered very rarely in port towns. The clinical
picture of soft-chancre ulcers is described in the respective section.
Thus, the diagnosis of the primary period of syphilis is made on the grounds of the clinical picture of primary
syphiloma and regional scleradenitis and obligatory identification of T. pallidum in the secretions of the chancre or
the aspirate of regional lymph nodes. The findings of blood tests for serological reactions (in the primary
seropositive period) and the results of confrontation (examination of the individual by whom the patient claims he
might have been infected) help greatly in the diagnosis.
Besides, the exacerbation reaction, or the Lukashevich-Jarisch-Herxheimer reaction develops after the
diagnosis of primary syphilis has been made and specific treatment (with water-soluble penicillin, as a rule) is
begun; it also confirms the correctness of the diagnosis retrospectively. It is believed that this reaction results from
mass disintegration of treponemas and the release of the endotoxin. The reaction is manifested by a chill, pain in the
muscles, bones and joints, and a rise in body temperature, sometimes as high as 40 C. The reaction occurs after two
or three penicillin injections and disappears in a few hours. In very rare cases the reaction may be so severe (a state
of dimmed consciousness, fever of more than 40 C, etc.) that one injection of penicillin has to be skipped in the
course. The frequency of the exacerbation reaction ranges from 49 to 84 per cent in primary seronegative syphilis
and from 58 to 94 per cent in the primary seropositive period.
Therefore, the modern course of primary syphilis is marked by the following peculiarities: frequent
prolongation of the incubation period, increase in the number of ulcerous and multiple chancres, which are often
complicated by secondary infection with signs of phimosis; the absence of marked induration in the base of the chan-
cre in individual cases, and a certain increase in the incidence of extragenital chancres. Besides, an inadequate
reaction of the lymphatic apparatus is often encountered now in the form of unilateral affection of the regional lymph
nodes, or absence of scleradenitis, increased incidence of the exacerbation reaction, a somewhat accel erated negative
Wassermann's reaction and, finally, earlier appearance of the signs of early secondary syphilis.
SECONDARY PERIOD OF SYPHILIS (SYPHILIS SECUNDARIA)
The secondary period of syphilis often begins with prodromal phenomena usually preceding secondary
syphilids by 7 to 10 days. They are mostly encountered in females or weakened patients and coincide in time with
the dissemination of treponemas in the patient's body by the haematogenous route. There are weakness, diminished
working capacity, adynamia, headache, pain in the muscles, bones and joints (intensified at night, which is
characteristic of syphilis);body temperature elevates (to moderate values, less frequently to 39- 40 C). This
condition is often mistaken by the patient and physician for influenza, which delays timely diagnosis of syphilis.
Blood leucocytosis and anaemia may be encountered in this period. The prodromal phenomena, which happen,
but not in all patients, disappear with the appearance of the symptoms of the secondary period of syphilis, as a rule.
The secondary period of syphilis is characterized by a wide variety of morphological lesions localized on the
skin and visible mucous membranes, as well as by (to a less extent) chang es in the internal organs, nervous system
and motor apparatus. The motor changes have no specific features, as a rule, and should rather be referred to the
body's reaction to the generalized infectious process. This period is divided into secondary early syphilis (syphilis II
recens), when the eruptions appear for the first time, and secondary recurrent syphilis (syphilis II recidiva) when the
eruptions reappear after an interval. The secondary period of syphilis develops2.5-3, less frequently 4 months after
infection. In non-treated cases recurrences may be suffered several times within 2-4 years and more. The diagnosis
of secondary latent syphilis (syphilis II latens) is made in the interval between the eruptions. The appearance and
disappearance of the eruptions in the secondary period, as well as their number and morphological character are
linked with the periods of the activity of the treponemas and their immunological interrelationship with the patient's
organism.
The common features of the eruption in the secondary period (secondary syphilids) are its appearance over the
entire body, rounded contours and discrete boundaries of the lesions, absence of a tendency to coalesce, a rose-red
colour with a bluish hue, absence of subjective disturbances and a benign character of the lesions (even if not treated,
they disappear some time later without a trace). A vast number of treponemas are found on the eroded surfaces of the
secondary syphilids (this applies in particular to eroded papules of the genitalia and oral cavity and condyloma
latum) because of which they are very contagious both during coitus and in close everyday contact. Serological
blood tests (Wassermann's and precipitin reactions) are sharply positive in almost 100 per cent of cases with
secondary early syphilis (with a high reagin litre of 1:160, 1:320) and in 96-98 per cent of patients with secondary
recurrent syphilis (in which case the reagin titre is lower). The blood immunofluorescence test (IFT) is sharply
positive in almost 100 per cent of cases. The T. pallidum immobilization test (TIP) is positive in almost half the
patients with secondary early syphilis (40-60 per cent immobilization) and in 60-80 per cent of patients with
secondary recurrent syphilis (70-90 per cent immobilization). Up to 50 per cent of cases with secondary recurrent
syphilis are attended with abnormalities in the cerebrospinal fluid with no clinical picture of meningitis (the
condition is called latent syphilitic meningitis).
The beginning of antibiotic therapy in patients with secondary syphilis (the secondary early form in particular)
is often attended with the exacerbation reaction (Lukashevich-Jarisch-Herxheimer reaction), which is manifested not
only by elevated body temperature and other general symptoms as the case is in primary syphilis, but by an increased
number of lesions and intensification of their colour. In some patients who begin treatment in the primary sero-
positive period of syphilis, the first penicillin injection causes the appearance of rose-coloured spots on the trunk,
obliging the physician to change the diagnosis to secondary early syphilis (the attending and on-duty physicians must
therefore examine the skin continuously with particular care after the beginning of specific treatment started in the
primary seropositive period of syphilis). According to Skripkin and others, the exacerbation reaction is encountered
in 70 per cent of females and in 80 per cent of males with secondary early syphilis and in only 10-20 per cent of
patients in secondary recurrent syphilis.
Histological examination of syphilids of the secondary period reveals perivascular infiltration (of various
degrees) consisting of plasma, lymphoid, epithelioid and occasional giant cells. Plasma cells predominate which
permits the syphilitic infiltrate of the secondary period to be called plasmosis.
The secondary syphilids are vascular spots (roseola), papules and, less frequently, vesicles and pustules.
Pigmented syphilids (syphilit-ic leucoderma) and syphilitic alopecia are also lesions of the se-condary period. True
pleomorphism is often encountered in patients when there are simultaneous eruptions of different morphological
lesions (e.g. rose-coloured spots and papules) or false (evolutional) pleomorphism when similar morphological
lesions (e.g. papules) are in different developmental stages.
The syphilids of the secondary early period are smaller, abundant, brighter in colour; they are localized
symmetrically, mainly on the skin of the trunk, show no tendency to coalesce and do not undergo scaling as a rule.
Remnants of the hard chancre and marked regional scleradenitis may be found during this period in 22 to 30 per cent
of patients. Besides, polyscleradenitis is more manifest (enlarged, hard elastic, mobile, painless axillary,
submandibular, cervical, cubital lymph nodes, etc.). Polyadenitis is encountered in 88 to 90 per cent of patients with
secondary early syphilis.
In secondary recurrent syphilis, the lesions are larger, less abundant and are often arranged asymmetrically.
They tend to form groups (figures, garlands, arches) and are paler in colour. The lesions are often localized in the
perineum, inguinal folds, mucous membranes of the genitals and mouth, i.e. in places subject to irritation. A
monomorphic eruption of rose-coloured spots is encountered in 55-60 per cent of patients with secondary early
syphilis; in secondary recurrent syphilis, in contrast, it is observed less frequently (in approximately 25 per cent of
patients); monomorphic papular rup-tion is encountered more often (in up to 22 per cent of cases).
The incidence of transient forms in which symptoms of early and recurrent secondary syphilis are found
simultaneously in a patient has greatly increased during the last decades. For instance, a small number of grouped
papules are found on various areas including the palms and soles (symptoms of the recurrent period) and remnants of
primary syphiloma, regional scleradenitis and polyadenitis (symptoms of the early period of secondary syphilis).
These cases are interpreted as protracted secondary early syphilis or as early recurrent syphilis (the latter diagnosis is
preferable from the standpoint of the prescription of a more intensive specific therapy). Skripkin and other authors
encountered such transient forms in 12.5 per cent of females and 5 per cent of males with secondary syphilis. It is
believed that secondary recurrent syphilis is encoun-tered more frequently than secondary early syphilis.

Macular Syphilid, or Syphilitic Roseola (Syphilis Maculosa, Roseola Syphilitica)

Macular syphilid is the most common morphological lesion of secondary syphilis, the early form in particular.
It is a rosy-red vascular spot with a bluish hue, which disappears when pressed. It is round, not elevated above the
skin surface as a rule, and reaches the size of the little-finger nail. No scaling occurs and no traces are left when these
spots resolve. The roseola is usually found on the sides of the trunk, the chest, abdomen and the upper limbs (it never
appears on the face, feet or hands). The spots develop gradually for one or two weeks, remain unchanged for one to
three weeks, and then turn pale and disappear.
Deviations may sometimes be encountered from the common clinical picture of roseola described above. The
spots, for instance, may be elevated above the skin surface permanently (elevated roseola) or temporary (urticarial
roseola). In the latter case the patient may experience itching and a sensation of burning and scaling may be found
when such spots resolve. In some cases the spots may coalesce (coalescent roseola), which is encountered mostly in
children, or may form rings (late roseola developing in late recurrences r even in the tertiary period of syphilis).
Granular roseola has bee described in individuals suffering simultaneously from syphilis and tuberculosis, and
haemorrhagic roseola in patients with increased permeability of the vascular walls.
Syphilitic roseola has to be differentiated at times from macular eruptions in certain infectious diseases and
dermatoses. Roseola may develop after medication with various drugs or after eating spoiled food (toxicoderma).
The spots of toxic roseola are brighter, tend to coalesce, follow a more acute course and are often attended with a
sensation of burning and itching; scaling develops ultimately. Properly taken medical history and the results of
serological examination of the blood for syphilis help in making the correct diagnosis.
The macular eruption in Jibert's pityriasis is also rose or pale rose with a yellowish hue. Scaling occurs in the
centre of the spots arranged as a long diameter on the lines of skin tension and one of the spots is larger than the
others (maternal patch). The patient experiences mild itching.
The rosela in typhus and enteric fever is always attended with severe general symptoms. The eruption is not as
abundant as in secondary early syphilis in which the general disorders if they had developed in the prodromal period
disappear with the appearance of the rose-coloured spots.
Spots resulting from the bites of lice (maculae caeruleae) do not disappear on being pressed by the finger; they
are attended with severe itching, have a bluishred or brownish colour, and are localised in places inhabited by the
parasites.
In pityriasis versicolor the colour of the spots may range from pink and light-brown to dark-brown, and scaling
occurs (they are easily differentiated by the iodine test).

Papular Syphilid (Syphilis Papulosa)


Papular syphilid is a frequent manifestation of the secondary, especially the recurrent, period of syphilis. The
papules are localized in the dermal papillary layer and may differ in size. Papules the size of a lentil (lenticular
papules) are encountered more frequently, those the size of a pinhead (miliary papules, or lichenoid syphilids) are
rarer. Lenticular papules are semispherical and have discrete boundaries and regular outlines; they are ham-coloured
and do not coalesce. Scaling is often encountered and the scales are arranged the periphery of the papule (Biett's
collar). Miliary papules are arranged around the orifices of the hair follicles and are mostly found in weakened
patients suffering, for instance, also from tuberculosis. Large papule's' with a diameter of about 10 to 20 millimetres
are called nummular papules. Under the effect of rubbing and irritation with various secretions papules localized in
the skin creases, around the anus and on the genitals may grow in size (hypertrophic papules). When they coalesce,
plaque-like papules or condylomata lata form (Fig. 35). These condylomata, h'ypertrophic papules and papules
found in the skin creases often undergo, erosion (erosive papules) as a result of rubbing, and oozing occurs (moist
papules). The secretions of the erosive and moist papules and the condylomata lata contain treponemas in
abundance. Such patients are therefore highly contagious and hazardous as a source of infection. Papular syphilid of
the palms and soles (palmar-plantar syphilid) is very peculiar in appearance: the papules are not elevated above the
skin surface but resemble congestive-red spots covered with clusters f hard horny scales. On palpation, however,
the spots are found to be hard. The papules may coalesce sometimes and form patches of various size with discrete
boundaries and hard horny masses on the surface.
In most cases the miliary and nummular papules are manifestations of the recurrent period of syphilis. The
lenticular and erosive papules are encountered both in secondary early and in secondary recurrent syphilis. The
lenticular papules may have an abundance of silver-white scales on their surface and thus resemble psoriatic papules
(psoriasiform syphilid); when arranged on the face or scalp in patients with seborrhoea oleosa they are covered with
oily yellowish scales (seborrhoeic syphilitic papules). Syphilitic papules leave intensive pigmentation which
disappears gradually.
Histological examination in cases with papules in the dermis reveals an inflammatory infiltrate composed
mainly of plasma cells arranged like a muff around the vessels as well as diffusely (in the papillary layer). Marked
hyperplasia of the vascular endothelium is seen.
Differential diagnosis. The absence of the terminal film, punctate haemorrhage and no tendency of the papules
to coalesce and form patches distinguishes psoriasiform syphilitic papules from psoriatic papules. It is more difficult
to differentiate seborrhoeic syphilitic papules and the common papules of seborrhoea. Other symptoms of syphilis
must be taken into account in such cases (e.g. roseolar-papular syphilid of the trunk or limbs, the results of blood
serological tests, the medical history and, finally, the results of specific treat ment, particularly in different cases in
which syphilitic and seborrhoeic papules are combined in patients suffering simultaneously from syphilis and
seborrhoea oleosa).
The papules of lichen ruber planus differ from the syphilitic papules by their polygonal shape, characteristic
lustre, a central umbilication, a tendency to form patches and by itching, which is very intense in some cases.
Isolated miliary papular syphilid resembles lichen scrofulosorum very closely. The latter, however, is mostly
encountered in childhood and adolescence. The diagnosis is made easier by the results of general examination for
tuberculosis and syphilis. Hypertrophic papules and condylomata lata have to be differentiated from (1) condylomata
acuminatum (which have a thin pedicle and do not have a hard infiltrated base), (2) pemphigus vegetans (soft
succulent vegetation covered with seropurulent and haemorrhagic crusts and surrounded by strands of epidermis or
fresh bullae), and (3) haemorrhoids (tendency to bleed, smooth surface, pain). The patches of palmar-plantar
syphilids have to be differentiated from palmar-plantar psoriasis, which proves to be extremely difficult at times. The
papular syphilid on the palms and soles has on its periphery a narrow ring of violet infiltrate covered with horny
masses. Besides, one should take into account the other symptoms of syphilis (e.g. the results of Wassermann's
reaction) and psoriasis (e.g. the frequent involvement of the nail plates , psoriatic onychia).

Pustular Syphilid (Syphilis Pustulosa)


Pustular syphilid is much rarer than macular or papular syphilid. It develops in weakened patients and in
individuals abusing alcohol. It is predominantly localized on the scalp, in the small of the back, and on the legs.
Pustular syphilids are often combined with papular syphilids. Several variants of pustular syphilids are dis-
tinguished which must be differentiated with pustular lesions, main-ly those caused by staphylococcal flora.
In impetigo syphilitica a pustule forms in the centre of the papule and rapidly dries to a crust. The absence of a
tendency of the lesion to peripheral growth and coalescence, the absence of subjective dis-turbances, the information
gained from the medical history, the results of serological blood tests, and other clinical manifestations of the
secondary period of syphilis enable the physician to make a diffe-rential diagnosis with impetigo vulgaris.
Acne syphilitica resembles acne vulgaris clinically. In making the diagnosis one should take into account the
absence of seborrhoeic phenomena (comedones in particular), localization of the eruption also on areas not typical of
seborrhoea, and other symptoms of the secondary period of syphilis.
Varicella syphilitica occurs in weakened patients as a rule and is characterized by the formation of a spherical
pustule the size of a small pea. The centre of the pustule dries very rapidly to a crust and is retracted (in this the
morphological lesion resembles the small-pox pustule), and a swelling of brownish-red infiltration forms around the
lesion. There are usually a few lesions (10-20) and the process lasts a considerable length of time (5-7 weeks) as a
rule and leaves no scars. Smallpox and chickenpox, with which a differential diag-nosis has to be made, are
characterized by an acute course, the severe general condition of the patient, the absence of an infiltrate around the
pustules, by an abundance of lesions and the absence of other symptoms of secondary syphilis, a negative blood test
for Wassermann's reaction among others.
Ecthyma syphilitica is a rare but severe manifestation of pustular syphilid. There are usually a few lesions (up
to ten). They are prevalently localized on the anterior aspect of the legs, less frequently on the trunk, limbs and scalp.
Ecthyma syphilitica is considered to be evidence of a malignant course of syphilis and occurs in weak-ened
individuals suffering from tuberculosis and in persons abus-ing alcohol; it develops no earlier than six months after
infection. The clinical picture is marked by a deep pustule, covered with a thick greyish-brown crust, as if pressed
into the skin (Fig. 36). An ulcer forms under the crust leaving a smooth scar after healing. A hard copperred infiltrate
forms on the periphery of the lesion, which does not occur in ecthyma vulgaris. The favoured 1lization of
echtyma syphilitica is the anterior aspect of the legs, whereas the comma forms of ecthyma are usually found in the
small of the back and the buttocks. Besides, a diffuse redness is encountered around the lesion in ecthyma vulgaris;
it may, however, also be found in ecthyma syphilitica complicated by secondary infection. The diagnosis is also
difficult to make because in ecthyma syphili-tica the serological reactions are often negative as the result of the
body's anergy.
Rupia syphilitica is a variant of ecthyma in which a layered co-nical crust forms. Peripheral growth of the
lesion and the presence of a large fleer under the crust are characteristic features. Rupia develops no earlier than a
year after the onset of the disease and, like ecthyma, is evidence of the malignant course of syphilis in the patient.

Secondary Syphilids of the Mucous Membranes


Secondary syphilids of the mucous membranes are quite common and because of the fragility of the tegmental
epithelium and frequent maceration are very hazardous as regards transmission of infection not only by the sexual
routes, but by close everyday contact. The oral cavity, throat, larynx, vermilion border and mucous membranes of
labia majora and minora are most frequently involved. Macular, papular and pustular syphilids occur here, just as on
the skin. Most syphilologists admit the possibility of syphilitic roseola forming on the mucous membranes, but pink
colour of the mucosa makes this eruption practically indistinguishable. The large erythematous areas of coalesced
roseolar lesions on the tonsils and soft palate (erythematous, syphilitic tonsillitis) may be localized both unilaterally
and bilaterally and there are no attendant subjective sensations, general symptoms or increased body temperature as
a rule. Moreover, the discrete boundary of the erythematous foci and their bluish shade make it possible to
differentiate the condi-tion from catarrhal tonsillitis of streptococcal aetiology. Skripkin states that erythematous
tonsillitis as a manifestation of mucosal macular syphilids is encountered in 47 to 55 per cent of patients suffering
from secondary syphilis.
Syphilitic papules are the most common lesions of the mucous membranes. Lenticular papules prevail. They
are flat, round, intensively dark red, hard to the touch, sharply demarcated and slightly elevated above the skin
surface. Papular tonsillitis develops in 12 to 15 per cent of patients with secondary syphilis. Because the epithelium
of the oral mucosa is macerated, the papules acquire a whitish opal colour with a dark-red ring on the periphery and
then undergo erosion; the papules coalesce quite often. The soft palate, lips, the mucous membrane of the tongue
(mostly its edge) and gums are the sites of oral syphilitic papules besides the tonsils. The mucosal papules usually
cause no subjective sensations, but when they undergo erosion and secondary infection occurs, tenderness develops
and a hyperaemic zone forms around them. In localization of the papules in the larynx, on the vocal cords, the voice
becomes hoarse (raucedo) and, in rare cases, total aphonia develops. Differential diagnosis is made with various
forms of tonsillitis, faucial diphtheria and Vincent's tonsillitis, which are characterized by a sudden onset, acute
course', pain, and severe systemic phenomena. Ulcers, and erosions in aphthous stomatitis are marked by sharp
tenderness absence of infiltration, by intensive hyperaemia around the r-sions, an acute onset and, at times, by
strands or remnants of bullae forming a ring around the erosions. It is sometimes difficult to differentiate papules
and patches of lichen ruber planus localized in the mouth, particularly if they form on the tongue mucosa. In such
cases the results of serological blood tests are considered as well as the character of the lesions, if such are present on
other body areas (the skin of the trunk, limbs, genitals). Pustular syphilids are no longer encountered on the mucous
membranes.

Syphilitic Alopecia, or Baldness (Alopecia Syphilitica)


Syphilitic alopecia may be microfocal, diffuse or mixed. Microfocal alopecia (alopecia areolaris) is
characterized by the appearance on the scalp of small round foci of thinning hair without inflammatory phenomena
or scaling (Plate V). A few months later the hairs grow again. In diffuse alopecia the hairs fall out regularly over the
entire scalp, though mostly on the temples. Syphilitic loss of hair is encountered in 15 to 18 per cent of patients with
secondary syphilis, mostly in those with secondary recurrent syphilis. Syphilitic alopecia in the region of the beard,
eyebrows and eyelashes (Pinkus's sign) occurs much less frequently. Microfocal alopecia should be distinguished
from alopecia areata celsi in which there are usually only a few foci, but they are larger and show a tendency to peri -
pheral growth and coalescence. It also has to be distinguished from superficial trichophytosis and microsporosis of
the scalp in which the hairs break off and scaling develops in the foci of affection. Dif fuse syphilitic alopecia has to
be differentiated from diffuse alopecia seborrhoeica. Other signs of secondary syphilis and the results of serological
blood tests for syphilis help in recognizing the disease.
Syphilitic Leucoderma, or Pigmentary Syphilid (Leucoderma Syphiliticum)
Pigmentary syphilid appears in patients no earlier than 5 to 6 months after infection, i.e. in the secondary
recurrent period. Whitish, as if depigmented, round or oval spots resembling lace-work or a net, form on
hyperpigmented skin on the side and back of the neck, in the axillae, and on the sides of the chest. Abnorma lities are
found in the cerebrospinal fluid, as a rule, but the available methods of examination yield no convincing data on
clinical affection of the nervous system of most patients with syphilitic leucoderma. Leucoderma is sometimes
attended with alopecia (in 8 per cent of patients with recurrent syphilis). During World War II, when living
conditions sharply deteriorated, leucoderma and alopecia were encountered much more frequently. In 1942-43, for
instance, leucoderma was found in 68 per cent of patients suffering from recurrent syphilis, whereas in pre-war years
it was encountered in only 20 per cent of cases. In recent years leucoderma is observed in 10 to 15 per cent of
patients, in 98 per cent of whom it is localized on the neck. It disappears in a few months, often independently of
specific treatment.
Thus, the course of secondary early syphilis is characterized by the presence of pleomorphic eruptions with
predominance of roseolar and papular syphilids, toxic roseolas (granular and haemorrhagic), earlier appearance
and increase in the number of palmar-plantar syphilids, deficient reaction of the lymphatic apparatus, a ra-ther high
percentage of the remnants of hard chancre and regional scleradenitis, increase in the number of transient forms and
a high incidence of the exacerbation reaction.
Besides its prevalence over secondary early syphilis, secondary recurrent syphilis has the following features: a
predominantly pleomorphic character of efflorescence, relatively rare occurrence of pustular syphilids, a certain
increase in the number of cases with syphilitic alopecia, earlier appearance and noticeable increase in the number of
palmar-plantar syphilids, preservation of polyadenitis, the presence, in some patients, of remnants of hard chancre
and regional scleradenitis and increase in the incidence of transient forms which complicates the differential
diagnosis of secondary recurrent and early syphilis. The extremely rarely encountered signs of a malignant course of
secondary syphilis are evidence that the syphilitic infection in recent years tends to be benign.

Affections of the Internal Organs, Nervous System and Motor Apparatus


Such affections are infrequent but they may be very diverse and follow a benign course, as a rule.
A picture of acute hepatitis with jaundice resembling Botkin's disease (infectious hepatitis) is quite a rare
occurrence. The liver is enlarged, tender and its function (pigmentary, antitoxic, carbohydrate, etc.) is disturbed.
Splenomegaly is also encountered in fairly many cases. The serological blood tests in these patients are sharply
positive, as a rule, which with the other symptoms of syphilis helps in recognizing the aetiology of hepatitis.
Symptoms of involvement of the kidneys may be found at the same time as the affection of the liver. They
include benign proteinuria manifested only by the presence of protein in the urine (up to 0.1-0.3 pro mille) and
syphilitic lipoid nephrosis.
Cardiovascular involvement in the secondary period may occur in the form of syphilitic myocarditis (mild
fatiguability, general weakness, dyspnoea), the diagnosis of which is made by means of electrocardiography.
Syphilitic gastritis (nausea, regurgitation, loss of appetite, re-duced gastric-juice acidity) and dry syphilitic
pleurisy are also rarely recorded.
Affections of the nervous system in the secondary period of sy-philis are encountered in the form of latent
syphilitic meningitis, acute generalized meningitis, subacute (basilar) meningitis, and syphilitic hydrocephalus.
Disturbed sleep, irritability, short temper, and a sensation of hunger are observed in some patients with
secondary syphilis.
If specific therapy is begun in good time, the affections of the nervous system and internal organs in the
secondary period respond adequately to treatment, to penicillin therapy in particular, and regress quite rapidly.
Pain in the bones is among the most frequent manifestations of affection of the motor apparatus at the end of
the primary and beginning of the secondary periods of syphilis. It is prevalently felt in the long tubular bones of the
lower limbs and usually intensifies during the night. This pain is called dolores osteocopi nocturni and is not
attended with any objective changes in the bones. Periostitis and osteoperiostitis marked by excruciating pain at
night are less frequent in the secondary period. They are prevalently localized on the tibia and skull.
Specific therapy leads to rapid resolution of affections of the motor apparatus of syphilitic origin.
TERTIARY PERIOD OF SYPHILIS (SYPHILIS TERTIARIA)
The tertiary period of syphilis develops in a small number of patients who were neglectful of treatment for
syphilis or were not treated at all. Old and very young age, traumas (physical, psychic, medicamentous), chronic
diseases and toxicosis, and alcoholism are conducive to the development of tertiary syphilis. This period of syphilis
usually begins three or four years after infection; in the last decades, however, it has shown a tendency to develop in
eight to ten years, and may even become manifest only in tens of years. Patients with tertiary syphilis are a rare
occurrence in the Soviet Union. In tsarist Russia they were often registered, particularly in rural areas.
In distinction from the secondary period, the clinical manifestations in tertiary syphilis are prevalently local,
often with involvement not only of the skin and mucous membranes, but of the internal organs, nervous system and
motor apparatus; a scar remains after the lesions resolve. The pathoanatomical essence of tertiary syphilids is an
infectious granuloma whose localization in some cases leads to impaired function of the organ in which it has
formed. The tertiary period of syphilis is subdivided into active tertiary syphilis and latent tertiary syphilis.
Involvement of the skin and mucous membranes (tertiary syphilids) may be displayed by tubercles or gumma
(gummata) on the skin, in the subcutaneous tissue, muscles, bones, internal organs and nervous system. Only a
number of the lesions form (tubercles form in dozens while gummata are usually solitary). They follow a malignant
course nd always leave scars (if localized in vitally important organs they create a threat to the patient's life). If no
treatment is applied, the lesions heal very slowly. They are not very contagious (treponemas are few in number and
contained deep in the infiltrate) and respond well to antisyphilitic treatment, particularly with iodine prepara tions and
salts of heavy metals. It should be borne in mind that in the tertiary period standard serological reactions are negative
in 25 to 35 per cent of patients. The results of blood tests for T. pal. lidum immobilization, which are very rarely
negative in the tertiary period, are of immense diagnostic value in such cases.

Tubercular Syphilid (Syphilis Tuberculosa)


Tubercular syphilid is usually localized on a small skin area, asymmetrically as a rule. The tubercle is
semispherical or flat, copperred with a cyanotic hue, and the size of a cherry stone. It is hard and has discrete
boundaries. The infiltrate of the syphilitic tubercle undergoes necrosis either of the dry type or with the for-mation of
ulcers. In the first case atrophy remains after the tubercle resolved. In the second case the ulcers leave a slightly
retracted focus of grouped scars surrounded by a pigmented ring. The tubercles erupt in bouts and are therefore
found in different stages of re-solution. As a result a 'tesselated scar' forms (particularly often after a grouped
tubercular syphilid) which many years later con-firms the fact that the patient had suffered from tertiary tubercu-lar
syphilis. Most frequent occurrences are grouped tubercular syphilid, in which the tubercles are arranged in a cluster,
or group without coalescing, and serpiginous (creeping) tubercular syphilid, in which the tubercles coalesce, heal in
the centre while new tubercles appear on the periphery of the focus. Tubercular syphilid, in which the tubercles
coalesce into a single patch, and dwarf syphilitic tubercles resembling a millet grain in size are encounered less
frequently. Tubercular syphilid causes no subjective disorders.
Tubercular syphilid has to be differentiated first of all from lu-pus vulgaris in which the tubercles (lupomas)
are soft (because of this the 'probe' phenomenon or Pospelov's sign is positive, i.e. the lupoma is easily pierced with a
blunt probe or its soft tissue is easily pitted) and coloured light-red with a yellowish tinge well demonstrated in
diascopy ('apple-jelly' phenomenon, 'apple mousse sign). In lupus vulgaris the tubercles are usually flatter, only
slight-ly elevated above the skin surface, and tend to coalesce into foci of extensive affection with ultimate formation
of a vast depigmented atrophic scar, in the thickness of which new lupomas often develop. When the lupomas
necrotize, ulcers with irregular and eroded contours form, they are reddish-cyanotic with soft irregular edges and
often with a granular floor. Lupus vulgaris lasts several years. The syphilitic tubercles are hard, ham-red or
intensively red in colour and are usually grouped in a focus without coalescing. If the tubercles in syphilis ulcerate,
the ulcers have regular round contours, sloping uneroded edges, a clean and smooth floor and a dense-elastic
infiltrate in the base. The scars remaining after syphilitic tubercles are mottled in colour because of irregular
pigmentation and uneven in relief ('tesselated scars'); new tubercles never form on them. The disease lasts weeks or
months, rarely longer.
Serological reactions, which are positive in some patients, as well as the T. pallidum immobilization and
immunofluorescence tests, which are positive in most patients, and, finally, the effica-cy of trial antisyphilitic
treatment help in recognizing tubercular syphilid.
A differential diagnosis has to be made less frequently between syphilitic tubercles and tubercles developing in
cutaneous leishmaniasis (Borovsky's disease), which are characterized by a yellowish colour, a pasty or moderately
dense consistency, and the presence of nodular lymphangitis on the periphery of the foci; the medical history
(development of the disease in zones endemic for leishmania-sis) and the results of microscopy of the discharge
(discovery of Borovsky's bodies) are taken into consideration.
Syphilitic Gumma (Gumma Subcutaneum), or Gummatous Syphilid
A painless nodule the size of a walnut forms in the subcutaneous tissue without adhesion with the skin or
surrounding tissues. It grows gradually, fuses with the skin, and loses its mobility. The skin becomes cyanotic-red
and tenderness appears. The node softens in the centre and a small opening forms from which a gummy fluid
resembling gum arabic (hence the name 'gumma') is discharged. The opening grows and an ulcer with hard edges
steeply inclined to the floor forms. Necrotic tissue, the gummatous core, is found on the floor of the ulcer; it
separates slowly after which the ulcer cicatrizes. A deeply-seated retracted (stellate) scar forms. Much less frequently
the gumma may resolve without the formation of an ulcer. The patient usually has one, rarely more gummata. he
gummata usually form on the anterior surface of the legs, on the forehead, and forearms; sometimes fibrous
gummata, periarticular nodules, form (mostly around the knees and elbows). On resolution the gummatosus nodules
grow smaller to the size of hazel-nut and their infiltrate is replaced by fibrous tissue wich lends the the nodules a
cartilaginous hardness. They are very resistant to specific therapy.
Tertiary syphilids (gummata and tubercles) are often localized on the mucous membranes of the nose, soft
palate and uvula. In involvement of the nasal bones the bridge of the nose sinks (the nose becomes saddle back ) or
the bony part of the nasal septum is perforated. Because of perforation of the hard and soft palate and destruction of
the uvula the voice acquires a nasal quality and food gets into the nasal cavity. Gummatous osteoperiostitis (fig. 39)
or osteomyelitis usually occurs in the tibia and skull bones. The bone becomes thickened or infiltrate disintegrates
with the formation of an ulcer, after which the tissue cicatrizes.
With the formation of gumma or tubercular syphilis the histological picture is marked by an inflammatory
infiltrate of the type of infectious granuloma and pronounced changes of the blood vessels. In cases with gumma, the
infiltrate is found in the subcutaneous tissue and then spreads to the dermis; in cases of tubercular syphilid, it is only
found in the dermis. The infiltrate contains very many plasma cells, lymphocytes, histiocytes and a more or less
conside-rable number of giant and epithelioid cells. Foci of necrosis are detected in the gummatous infiltrates.
Proliferation of the endothe-lium is seen in the walls of the vessels, especially the large ones, which may lead to
obliteration of the walls.
The diagnosis is very difficult in the initial developmental pe-riod of the gumma, but much easier after its
ulceration (the char-acteristic clinical picture of gummatous ulcer) and in cases when the patient applies to the
physician with a formed characteristically stellate scar.
Syphilitic gummata are differentiated from scrofuloderma. In scrofuloderma the nodules are softer, undergo
total ulceration and the edges of the ulcer are soft and its floor is covered with yellowish granulations. A differential
diagnosis is also made with erythema induratum (usually manifested by multiple nodules arranged sym-metrically on
the legs, sometimes by superficial ulcers with eroded edges) and carcinomatous ulcers (hard, elevated, swollen or
'evert-ed' edges and an uneven easily bleeding floor).
Involvement of the internal organs, nervous system and motor apparatus in the late forms of syphilis, tertiary
syphilis among others (solitary gummata or diffuse infiltration), is described below.

LATENT SYPHILIS (SYPHILIS LATENS)


In his everyday practice the physician encounters patients in whom the diagnosis of syphilis is based only on
positive serologic-al reactions in the absence of any clinical manifestations (skin and mucosal lesions, symptoms of
involvement of the internal organs, nervous system or the weight-bearing, and motor apparatus) testi-fying to the
presence of a specific infection in the patient's body. Many authors cite statistical data according to which the
incidence of latent syphilis has increased in many countries. Latent syphilis, for instance, is revealed in 90 per cent
of patients during prophylac-tic examination, in women's consultation centres and in somatic hospitals. This is
explained by a more thorough examination of the population (i.e. improvement of diagnostics) and by the actual
increase in the number of patients (among the causes of this is the wide use of antibiotics by the population for
intercurrent diseases and manifestations of syphilis which the patient believes to be not symptoms of a venereal
disease, but manifestations of allergy, cold, etc.).
The group of patients sharing the diagnosis of latent syphilis is heterogeneous immunobiologically and
epidemiologically . According to the international classification of diseases, injuries and causes of death, latent
syphilis is subdivided into early and late forms; latent syphilis innocentum is also distinguished.
Early latent syphilis 'coincides in time with the period lasting from primary seropositive syphilis to secondary
recurrent syphilis inclusively, but there are no active clinical manifestations of the latter (two years from the moment
of infection, on the average). The patients, however, may develop active contagious manifestations of early syphilis
at any moment. This makes it necessary to refer patients with early latent syphilis to the epidemiologically hazardous
group and apply vigorous anti-epidemic measures (isolation of patients, thorough examination of sex partners and
everyday contacts, application of compulsory treatment whenever necessary, etc.). Just as the management of
patients with other early forms of syphilis, treatment of patients suffering from early latent syphilis is aimed at rapid
elimination of the syphilitic infection from the body.
The following information may be of assistance in the diagnosis of this form of syphilis:
(1) the medical history which should be taken thoroughly with proper attention focused on a past history (of
one or two years) of erosive and ulcerous efflorescences on the genitals and in the mouth, various eruptions on the
skin, on medication with antibiotics (for 'sore throat', 'influenza'), on treatment of gonorrhoea (without examination
of the source of infection), etc.;
(2) the results of confrontation (examination of the sex partner and detection of early forms of syphilis);
(3) detection of a scar or induration at the site of primary syphiloma and enlarged lymph nodes (usually
inguinal) corresponding clinically to regional scleradenitis;
(4) high reagin titre (1:120, 1:360) in sharply positive results of all serological tests (it may be low in patients
who had been treat-ed for gonorrhoea or who had indulged in self-treatment);
(5) a temperature exacerbation reaction at the beginning of penicillin therapy;
(6) rapid drop in the reagin titre as early as during the first course of specific treatment; the serological
reactions are reversed to negative by the end of the first to second course of treatment;
(7 ) sharply positive immunofluorescence test in these patients, although the T.pallidum immobilization test
may still be negative in some of them;
(8) the patient's age (the disease usually occurs in patients under forty);
(9) the cerebrospinal fluid may be normal (in latent syphilitic meningitis the cerebrospinal fluid is rapidly freed
of infection during antisyphilitic treatment). In accordance with existing instructions and schedules for syphilis
treatment, all patients with early latent syphilis are subject to the same therapy. The out-come of the disease may be
prognosticated when the duration of the infection can be determined either from the medical history or from the
findings of confrontation (it is natural that the shorter the duration of the disease, the more favourable are the
prognosis and the result of treatment).
Patients with late latent syphilis are not considered hazardous epidemiologicall. In such cases, however,
positive serological reactions of the blood may be particularly easily mistaken for a manifestation of syphilis,
whereas actually they may be pseudopositive, i.e. non-syphilitic, resulting from a number of conditions (malaria,
rheumatism, chronic diseases of the liver and lungs, chronic purulent processes suffered in the past, age metabolic
changes, etc.). The establishment of this diagnosis is considered to be most compli-cated and very serious and should
be verified by the immunofluorescence T. pallidum immobilization tests (in some cases these tests are repeated at
intervals of several months after foci of chronic in-fection are cured or after intercurrent diseases are treated).
The following information facilitates the diagnosis of late latent syphilis:
(1) the medical history (if the patient thinks that the infection might have been acquired from some source
more than two years previously);
(2) low reagin titre (1:5, 1:10, 1:20) in sharply positive results of the classical serological test (GST) or weakly
pos-itive results of CST (with confirmation of both cases by IFT and TPI);
(3) reversal of serological reactions to negative by the middle or end of specific treatment and the frequent
absence of negative reversal of CST, IFT and TPI despite vigorous antisyphilitic treat-ment and the use of non-
specific agents;
(4) absence of the exacerbation reaction at the beginning of penicillin therapy; it is pref-erable to begin
treatment of such patients with preparatory agents such as iodine preparations and bioquinol (quinine in neutral
vege-table oil); (
5) abnormalities in the cerebrospinal fluid (latent syph-ilitic meningitis) which are encountered more often in
these pa-tients than in those with early latent syphilis and are corrected very slowly.
Moreover, the sex partners may also have late latent syphi-lis or (much more frequently) they may have no
manifestations of the syphilitic infection (they are practically healthy and should not be subjected to preventive
treatment as the sex partners of pa-tients with early latent syphilis). The main purpose of specific therapy for patients
with late latent syphilis is the prevention of late forms of visceral syphilis and syphilis of the nervous system.
The diagnosis of latent (unidentified, unspecified) syphilis is made when neither the physician
nor the patient knows when or under what circumstances infection had occurred. Such a diagnosis is now
encountered ever less frequently because latent syphilis is now divided into the early and late forms. The diagnosis is
of or latent syphilis innocentum made in cases with no his-tory or clinical findings of the disease confirms the
possibility of syphilis following an asymptomatic latent course from the begining.

AFFECTION OF BONES AND JOINTS IN SYPHILIS


Bone involvement in late syphilis has long been known. As early as 1544 Vigo reported about exostoses and
night pains in the bones of syphilitic patients, but detailed information on bone lesions in syphilis was only obtained
in the 19th century with the develop-ment mainly of pathological anatomy and histology.
The following main forms of bone affections in syphilis are distinguished: periostitis, ostitis and osteomyelitis.
They may be localized or diffuse, generalized. This subdivision of the processes is of relative significance because
they are usually combined (ostitis, for instance, is always accompanied with periostitis, whereas osteomyelitis is
actually ostitis but with extension of the process deep into the bones).
Syphilitic periostitis is rarely found in the bone alone; it is combined with ostitis. In those rare cases, in which
only the periosteum is involved, the process occurs simultaneously in many bones and is distinguished by its long
duration. Diffuse syphilitic periostitis is of a gross character and develops as a ridge or lacework and is therefore
called ridge-like or lace-like. It usually occurs on the anterior surface of the tibia. Gummatous periostitis is
predominantly a localized process, a manifestation of the bone reaction to the gumma. Periosteal gumma causes
destruction and a defect in the bone. Destruction results not only from pressure of the infiltrate on the bone tissue,
but is also caused by the inflammatory process spread-ing from the periosteum to the bone (this condition is called
gum-matous osteoperiostitis). Sclerosis of bone tissue and ossification of the periosteum occur around the defect.
Clinical examination reveals a hard delimited swelling which sometimes conspicuously protrudes above the surface
of the bone. Patients suffer pain which is intensified at night. Such gummatous lesions develop most frequent-ly on
the bones of the skull cap and anterior surface of the tibia (Fourniet called it 'the favoured bone of syphilis'). A
solitary gumma of the bone may be gradually replaced by connective tissue and ossified, but more often the
gummatous infiltrate disintegrates, the process extends to the skin overlying the diseased bone area, and a
characteristic deep gummatous ulcer forms with necrotic bone tissue on its floor.
The gummatous process often spreads over a considerable area of the bone, penetrates deeply into it and
through its entire thickness, including the bone marrow. Such a condition is called gummatous osteomyelitis.
Morphological destructive and productive pheno-mena take place in such a process with predominance of the latter.
The bone thickens and hardens. It becomes distorted and its edges become uneven. On X-ray these lesions resemble
other chronic inflammatory processes in the bone developing with a marked bone reaction. The specific gummatous
lesions are masked by the productive hyperostotic changes. The medical history, the other clinical manifestations of
syphilis, the results of laboratory tests (blood, cerebrospinal fluid), and in many cases the results of trial treatment
(therapia ex juvantibus) make the diagnosis easier.
The short bones (vertebrae, the carpal and tarsal bones) are very rarely involved, but it is still more difficult to
make the diagnosis in such cases because the gummatous changes of this localization are characterized by
destruction with poorly pronounced bone reac-tion around the focus. No periosteal stratifications or hyperostotic
phenomena are encountered.
The joints are involved in syphilis much less frequently than the bones. Two forms of syphilitic arthritis are
mainly distinguished; primary synovial (affection of the articular capsule and bursa) and primary osteal (affection of
the articular bones and cartilages). Primary synovial arthritis may be acute or chronic. Reactive arthritis, arising from
the effect of a gummatous process taking place in the vicinity of the joint (epiphysis, metaphysis) is most often relat-
ed to the acute form. Chronic synovial arthritis (usually considered to be of allergic origin) is encountered more
frequently. It is clinical-ly manifested by pain, spherical swelling of the joint, intra-articu-lar effusion, and mildly
impaired function of the joint. Osteoarthritis develops as the result of gummatous affections of the articular ends of
bones (gummatous epiphysitis). X-ray demonstrates round honeycomb defects in the epipnyses with a poorly
pronounced surrounding sclerotic reaction. The knee, shoulder, elbow and ankle joints are involved most frequently;
deformity develops gradually, but movements at the diseased joint are preserved and pain is mild; the patient's
general condition hardly changes. These signs, as well as other clinical and laboratory findings are taken into con-
sideration in making the differential diagnosis, with tuberculous arthritis in particular.

SYPHILIS OF THE INTERNAL ORGANS (LUES VISCERALIS)


Since it is an infection of the whole body, syphilis affects many internal organs and systems in its early stage of
development. In the primary, secondary and early latent syphilis, however, these affections in most cases are
functional in character, only slightly manifested clinically, unstable, and respond adequately to the effect of
antisyphilitic agents.
In the late forms of syphilis, tertiary syphilis included, gummatous processes may be encountered in different
internal organs as well as diseases which can be referred to as true visceral syphilis.
The cardiovascular system and liver are the internal organs most prone to involvement. Syphilitic myocarditis
of the secondary, and especially the tertiary, periods of syphilis is characterized by dyspnoea, rapid fatiguability,
malaise, arrhythmia, diminished heart sounds and expansion of the heart boundaries to the left in particular.
Syphilitic myocarditis has no specific clinical features and does not differ from myocarditis of other origin. Its
diagnosis is based on the other clinical and laboratory signs of syphilis, aorti-tis in particular, which often attends
myocarditis.
Syphilitic aortitis, is the most common manifestation of visceral syphilis. The main changes take place in the
middle coat of the aorta, in view of which the condition is called mesaortitis; it results in hardening of the aortic wall
and dilatation of the ascending aorta (this last sign is very pathognomonic of syphilitic affection of the aorta). The
ascending aorta normally measures 3 to 3.5 cm, in mesaortitis, in contrast, it is 5 to 6 cm and often has a flask-like
dila-tation. Syphilitic mesaortitis is often attended with insufficiency of the aortic valves caused by dilatation of the
fibrous valve ring and by occlusion of the orifices of the coronary vessels. Affection of the valves leads to the
appearance of diastolic and systolic murmurs and accentuation of the second aortic sound which sometimes has a
metallic ring. Accentuation of the second aortic sound with a metallic sound is pathognomonic for syphilitic
affection of the car-diovascular system. Such patients complain of dyspnoea, pain in the heart, and palpitation.
Syphilitic aortitis may lead to a dangerous complication, the development of aortic aneurysm, the rupture of
which causes immediate death in one third to one fourth of cases.
Affection of the liver in the tertiary period of syphilis is encountered in 2 to 4 per cent of cases with visceral
syphilis. Focal gummatous, miliary gummatous, or diffuse infiltrative, and chronic epithelial syphilitic hepatites are
distinguished. In the first case, gum-matous nodules of various size form in the liver, which cause its enlargement
and make it nodular (this is sometimes palpable). The patients may complain of pain in the right hypochondrium and
elevated body temperature (37-38 C, sometimes to 39 C). Jaun-dice develops rarely. The condition improves
rapidly under the efftect of specific treatment. Miliary gummatous hepatitis (is marked by the formation in the liver
of many small foci of gummatous structure, which cause irregular enlargement of the liver: jaundice does not
develop but there is moderate pain in the right hypochondrium A similar clinical picture occurs in diffuse gummatous
mfiltratioin around the blood vessels. If specific treatment is not begun in good time, fibrosis of the liver develops
(the liver becomes smaller, hardens, functional disorders occur, the patient feels indisposed, etc.) which may prove
fatal. In the very rarely encountered epithelial hepatitis fatal hepatic insufficiency develops in a few years and the
patient dies. In such cases the affection has no specific features and its syphilitic origin is established from other
symptoms of syphilis or the medical history.
The other internal organs (kidneys, lungs, stomach, intestine, etc.) are now very rarely involved in tertiary
syphilis (together they account for no more than 1.0 to 1.5 per cent of specific visceral pathology of the tertiary
period).

SYPHILIS OF THE NERVOUS SYSTEM (NEUROSYPHILIS)


Affections of the nervous system in the secondary period of syphilis ) are discussed in the respective section of
this textbook. Here we shall deal with those lesions of the nervous system, which are encountered in tertiary syphilis,
and mostly as an isolated independent manifestation of late syphilis with localization of the process in the vessels of
the brain or spinal cord, in the meninges (mesenchymal neurosyphilis) or in the brain matter (parenchymatous neufo-
syphilis)..
The principal form of mesenchymal neurosyphilis encountered currently is late meningovascular (lues
meningovascularis) or pure vascular neurosyphilis with predominant involvement of the cerebral vessels (lues
cerebri) or the brain and spinal cord (lues cerebrospinalis).
Mild headache, dizziness and tinnitus may be observed as a result of inflammation (usually chronic) of the
meninges. The main clinical symptoms, however, are associated with affection of various vessels of the brain or
spinal cord, in which a specific infiltrate is deposited. As a consequence, disturbances in the motor sphere (pareses,
in rare cases paralyses), sensory sphere (paraesthesia, disorders of tactile, temperature sensitivity, etc.), speech, and
psychic activity may occur. In many cases this condition has to be differentiated from hypertensive crisis in
individuals suffering from atherosclerosis. The diagnosis of syphilitic affection of the vessels may be prompted by a
relatively young age of the patient (although hypertension crises are sometimes encountered in young individuals),
normal arterial pressure values, usually a benign onset of the disease, remissions (improvement in the condition)
also occurring without treatment, 'scattered' symptoms (e.g. paresis of the right foot paraesthesia in the region of the
left forearm, slurring speech, etc )'. positive results of serological tests (particularly the TPI which in most cases of
vascular syphilis is positive, while the CST may be negative in more than 50 per cent of cases), abnormalities in the
cerebrospinal fluid in meningovascular syphilis (in pure vascular neurosyphilis the cerebrospinal fluid is normal), the
medical history in some cases and, finally, the results of ex juvantibus antisyphilitic treatment.
Late forms of syphilitic vascular lesions are now encountered in more than 70 per cent of cases with late
syphilis of the nervous system. Their timely diagnosis is very important because the correct diagnosis alters the
prognosis in a favourable direction (syphilitic affection of the vessels responds well to antisyphilitic treat-ment in
most cases which leads to total or almost total restoration of the function of the involved area).
Tabes dorsalis and general paresis are the main forms in involvement of the brain matter (parenchymatous
neurosyphilis). Tabes dorsalis characteristically produces very few symptoms now, and subjective disorders are often
absent (tabetic pain, crises, paraesthe-sia and disorders in the function of the pelvic organs are not encoun-tered).
This is why such patients rarely go to the doctor on their own initiative. They are detected by chance, as a rule,
during ex-amination by a neurologist or during treatment at a hospital for some other disease.
Difficulties are linked with the fact that in 40 per cent of patients the classical serological blood reactions may
be negative. The following clinical symptoms are very important in the diagnosis of tabes: pupillary disorders
(miosis, mydriasis, anisocoria and the Argyll Robertson pupil, which is particularly pathognomonic), loss of knee
and Achilles reflexes, swaying in Romberg's posture, tabetic dissociation, trophic disorders, cold hyperaesthesia in
the back, pain hypaesthesia in the chest (Hitzig's girdle), and others. Most of these symptoms, however, do not
trouble the patients and produce no effect on the function of different organs. That is why, as it was pointed out
above, the patients rarely apply for medical advice on their own initiative.
The diagnosis of general paresis is established, and the patients are put on record and treated by psychiatrists
for the most part. In some cases, however, particularly in the initial stages of the disease, the patients may be treated
by a venereologist (this applies mainly to individuals who had been treated for syphilis for a long time and
inadequately). It is important to suspect the disease; the diagnosis is eventually confirmed in 100 per cent of cases by
sharply positive results of CST and the TPI (90 to 100 per cent of immobilization) and the discovery of abnormalities
in the cerebrospinal fluid (Lange's test is characterized by a paralytic curve, e.g 6665433210). A change in the
patient's personality (unjustified change in character, behaviour and attitude to others), impairment (lapses) of
memory, disorders of speech, handwriting, counting, etc. are the initial clinical symptoms of the disease.

CONGENITAL SYPHILIS
General Information. Pathogenesis
As early as the end of the 15th and the beginning of the 16th centuries it was noted that syphilis could be
transmitted to the prog-eny. The mechanism of the transmission, however, remained disputable for several centuries.
Though even then some physicians believed that the foetus could be infected with syphilis only by a syphilitic
mother and that the infection is transmitted through the placenta, most physicians advocated the germinative
hypothesis, claiming that the syphilitic infection is transmitted to the foetus only by the father through a
spermatozoon infecting the ovum directly. It was assumed that the causative agent penetrated the spermatozoon
before or during fertilization because the semen was infected. According to this viewpoint, a syphilitic child may be
born of a healthy mother if the father has syphilis. This assumption was based on numerous clinical observations (the
causative agent of syphilis was still unknown at the time and a serological diagnosis of the disease did not exist):
long-term follow-up of many mothers who had given birth to children suffering from congenital syphilis revealed no
manifestations of syphilis.
It was believed that a foetus with syphilis immunizes the mother against the disease in the period of
intrauterine development so that she does not contract it when she suckles the infant. This premise is known as
Colles-Baumes' law. According to Profeta's law, which also confirms the germinative hypothesis of syphilis
transmission to the progeny, healthy children born of a mother suffering from syphilis remain unsusceptible to the
disease later, sometimes even till the period of puberty. In 1903 the Viennese scientist Matzenauer contributed
greatly to the substantiation of the theory of the ma-ternal, placental transmission of syphilis to the progeny on the
ba-sis of longterm clinical observations. Universal recognition, how-ever, was earned by this theory only after the
serological methods that a 'healthy' mother who gives birth to a syphilitic child, actually has latent ('serological')
syphilis and that many 'healthy' children born of syphilitic mothers are found to have latent congenital syphilis. For
instance, according to the Institute of Paediatrics, AMS USSR, Wassermann's reaction was positive in more than 90
per cent of mothers who had no clinical manifestation of syphilis but gave birth to children suffering from syphilis
(according to other sources this was found even in 97 to 100 per cent of cases). T. pal-lidum is detected in the body
of the foetus only in the second half of intrauterine life (when placental circulation begins functioning). In early
miscarriages in syphilitic women the foetus has no syphilitic affection. It is believed that treponemas enter the body
of the foetus either by the haematogenous (through the umbilical vein) or the lymphogenous (along the lymph slits of
the umbilical cord) route.
Congenital syphilis mortality rate is in inverse proportion to the child's age, that is why social and prophylactic
measures of congenital syphilis control acquire major importance. According to some statistical data, children
accounted for 20-25-35 per cent of all patients with syphilis in prerevolutionary Russia. In contrast, congenital
syphilis, especially the early form, is now an extremely rare occurrence in the USSR, which is one of the ma jor
achievements of Soviet public health and the venereological service in particular.
Congenital syphilis morbidity rate is still high in some other countries. It is considered possible that a mother
suffering from congenital syphilis (syphilis of the second and even third generation) may give birth to children with
syphilis. Such cases occur very rarely, however.
Pregnancy in a woman with untreated syphilis may terminate in late miscarriage or premature delivery of a
dead child or a child with early manifestations of congenital syphilis. A healthy infant may be born of a mother who
had received specific treatment (both before and during pregnancy) or of later pregnancies (the mother's property for
transmitting syphilis to the progeny diminishes three to four years after infection, but is not lost completely). The
longer the time from the moment of the mother's infection with syphilis, the less pronounced are the manifestations
of congenital syphilis.
The following forms of true congenital syphilis are distinguished: syphilis of the placenta, syphilis of the
foetus, infantile congenital syphilis, congenital syphilis of early childhood (all these forms are manifestations of
early congenital syphilis) and late congenital syphilis.
Some authors (Sukhareva, Gurevich, Milich, Sazonova, Yavkin and others) claim, however, that the syphilitic
infection may also influn the chromosomal apparatus of the parents' germ cells. Syphilitic gametopathy
(degenerative changes arising in the sex cell before fertilization), blastopathy (affection of the embryo in the
blastogenesis period), and syphilitic embryopathy (pathological changes in the foetus between the fourth week and
the fourth or fifth month of pregnancy) are distinguished. Various physical, neurological, and psychic, intellectual
defects are found in these sick children. Dystrophies of the bone system similar to stigmata of late congenital
syphilis are among the physical disorders. Organic and functional microsymptoms of affection of the nervous sys-
tem are the neurological disorders. The psychic disorders include oligophrenia of various severity, psychopathic-like
behaviour, en-cephalo-asthenic or schizophrenic-like syndrome, etc. The con-genital pathological manifestations in
these patients are not associated with the direct entry of T. pallidum into the body of the foetus and therefore cannot
be referred to true congenital syphilis. Since there is no causative agent in the child's body in such cases, the
serological reactions in blood and cerebrospinal fluid are always neg-ative. There are no abnormalities in the
cerebrospinal fluid, the TPI and I FT are negative, there is no advancement of the disease and antisyphilitic agents
have no marked therapeutic effect. Psy-chiatrists and some venereologists, however, contend that these damages
'would never have occurred' if the parents or progenitors had not suffered from syphilis. These lesions may develop
mostly in children whose parents had suffered or are suffering from late forms of syphilis. The clinical symptoms
and pathogenesis of these processes are still being studied. In all probability there are cases in which the symptoms
of true congenital syphilis occur in conjunc-tion with symptoms of parasyphilis. The latter is sometimes called
dystrophic syphilis. The diagram of the course of congenital syphilis is shown below.
Affection of the Placenta in Congenital Syphilis
Syphilitic affection of the placenta is manifested by oedema and proliferation of cell elements leading to an
increase in its mass and size. In the absence of abnormalities the correlation of the placenta mass and the foetal mass
is 1:6 or 1:5. Because of oedema and pro-liferation of cell elements this correlation changes to 1:4 or even 1:3.
Lesions of the vessels and central part of the villi are especially pro-nounced in the embryonal (foetal) part of the
placenta. Histologi-cal examination reveals endo-, meso- and perivasculitis and sclerosis of the villi. Syphilis of the
placenta is characterized by hyper-plasia of the connective-tissue cells (histiocytes, fibroblasts, etc.). i.e. proliferation
of granulation tissue, and the formation of abscesses in the vessels of the villi because of the presence of necrotic
foci resulting from obliteration of the vessels. These changes are usually not found in the maternal part of the
placenta. All these severe anatomomorphological lesions, however, cannot be regarded strictly pathognomonic of
syphilitic affection of the placenta. Detection of . pallidum in the umbilical cord and the foetal organ is of decisive
significance for the diagnosis. Because of the immunity phenomena in the mother's body treponemas are found less
frequent-ly in the placenta itself. Juice squeezed out of the placenta is exa-mined for treponemas, although, as
already indicated, the percent-age of their discovery is not great. Leucocytic infiltration of the vascular walls, most
marked in the umbilical vein, is found in the umbilical cord.
Syphilis of the Foetus
Damage to the foetus by the syphilitic infection is manifested by severe changes in the internal organs and
bone system. They are revealed no earlier than the fifth month of intrauterine life and are linked with mass
penetration of the placenta by treponemas. The greatest number of treponemas are found in the liver, spleen and
adrenals. The foetal parenchymatous organs affected with syphilis become enlarged and firm, a fact associated with
the develop-ment of diffuse proliferative infiltration and subsequent growth of connective tissue. Besides the liver
and spleen, the lungs, kidneys, pancreas and gonads also have similar diffuse inflammatory changes in their tissue.
The pronounced diffuse interstitial hyperplasia, copious desquamation of the alveolar epithelium, and growth of cells
in the interalveolar spaces in the lungs may give rise to a specific condition called white pneumonia.
Affection of several vitally important parenchymatous organs leads to the death of the foetus. In such cases
abortion occurs in the fifth or sixth months of pregnancy, or a stillborn child is delivered in the eighth month. The
macerated wrinkled and flabby skin makes the stillborn infant look old.
Clusters of cell elements are seen around the vessels or within the parenchyma of the affected organ (miliary
syphilomas) in some cases when the syphilitic process has a localized and focal character instead of a diffuse
inflammatory one. In such a case the child may be born alive, but it is poorly resistant to infections because the
defence forces of the body are weak; it dies (without treatment) within the first weeks or first months of life.
Intensified growth of bones usually takes place in the last months of intrauterine life in view of which the
bones are involved later than the parenchymatous organs, usually in the sixth lunar month. Grade I, II and III specific
osteochondritis of the long tubular bones develops often at the junction of the epiphyses and diaphyses where the
bones grow in length; osteoperiostitis occurs in some cases.

Congenital Infantile Syphilis


This form of congenital syphilis is manifested either from birth, usually in the first two months, or at the age of
two to four months. Diverse lesions of the skin, mucous membranes, bones, internal organs, nervous system and
sensory organs are encountered in this stage. The development of such children is retarded both in growth and
weight, they are weak, restless and hypotrophic. Their skin is wrinkled, dry and sallow. Hydrocephalus, periostitis of
the skull bones and dilation of the cranial veins are frequently observed.
A peculiar specific lesion is found on the skin of the face, palms, soles and buttocks, which is known as
Hochsinger's diffuse papular infiltration. The favoured sites of the process on the face are the skin around the mouth,
the chin, the forehead and the su-perciliary arches. This is the earliest (the end of the first and the be-ginning of the
second months of life) and the most common skin affec-tion in congenital infantile syphilis. Several spots or diffuse
erythe-ma appear first on the involved areas. The skin here becomes indu-rated and thickened and acquires a dark-
red colour. The involved areas are gradually covered with laminar scales. An extensive in-filtrate often forms; it is
pierced with deep fissures around the mouth because the infant cries and sucks. Radial scars remain for life in place
of the fissures (Robinson-Fourniet scars). The retrospective diagnosis of congenital syphilis may be made from them
many years later. Loss of hair, eyebrows and eyelashes occurs in infants if the diffuse infiltration formed of
coalesced papules involves the skin of the eyelids, superciliary arches and scalp. The syphilitic infiltrate in the nasal
mucosa narrows the nasal meatus, so that breathing becomes difficult, especially when the infant is fed at the breast.
Very many treponemas are found in the lesions of the diffuse infil-tration.
Syphilitic pemphigus is another early and peculiar sign of con-genital infantile syphilis. Vesicles the size of a
pea or len-til, with a seropurulent content, and surrounded by a ring of infil-tration form.
Tensed vesicles arranged symmetrically on an infiltrate base (usually on the soles and palms) are characteristic
of syphilitic pem-phigus. Somewhat less frequently the vesicles are localized on the flexor surfaces of the upper and
lower limbs, and still less frequently on the face and trunk. They do not tend to coalesce and there are do
accompanying subjective disorders. Many treponemas are easily detected in the contents of the vesicles. Syphilitic
pemphigus, like Hochsinger's infiltration, is a peculiar manifestation of early congenital syphilis and never occurs in
children with acquired syphilis.
Syphilitic papules are fairly often encountered in congenital infantile syphilis and resemble the lenticular
papules of the secondary period of acquired syphilis, but in distinction, they tend to coalesce. Brownish red spots
with mild infiltration of the base sometimes form on the trunk and limbs. They are later covered with small scales. A
typical roseola is a very rare occurrence in infants-The nasal mucosa is involved most frequently (syphilitic
rhinitis). This affection occurs in intrauterine life and is therefore found at the child's birth. Less frequently it
develops in the first month of life. Three stages of syphilitic rhinitis are distinguished. In the first (erythematous)
stage there are mild swelling and induration of the mucous membranes, which has no substantial effect on the
infant's condition. The second (secretory) stage is marked by a con-siderable swelling and copious purulent
secretion. This is the most commonly encountered stage. Nasal breathing becomes difficult, noisy and wheezing, or
the infant cannot breathe through the nose at all, as a result of which it is hard for him to suck at the breast and he
becomes emaciated. In the third (ulcerative) stage, which is rarer, destructive processes form and cause destruction
of the car-tilaginous and bony tissue of the nasal septum, which may lead to the formation of a saddleback, goat-like
or opera-glass nose. Syphilitic rhinitis is an especially important and valuable symptom of congenital syphilis
because it often precedes the other manifesta-tions and sometimes is the only symptom of syphilis in infants. It may
also be combined with other symptoms, affection of the bones and internal organs, in particular, T. pallidum is often
found in great numbers in the secretions of the nasal mucosa. Involvement of the laryngeal mucosa is much rarer, but
if this happens (diffuse, inflammatory infiltration), the voice becomes hoarse and aphonia, less frequently stenosis,
develop. Affection of the oral and faucial mucosa is still less frequent; erosions and superficial ulcerations develop in
such cases.
Bones are involved very often in congenital infantile syphilis (according to some authors in more than 80 per
cent of cases). Infants may have syphilitic osteochondritis, periostitis and osteoperiostitis. Syphilitic osteochondritis
of the long tubular bones (usually of the upper limbs) is most typical of congenital infantile syphilis. In the
initial stages (grade I or II osteochondritis) the process is identified by means of radiographs. Irregular expansion of
the zone of preliminary calcification (up to 2.0-2.5 mm; normally this is a regular clear strip up to 0.5 mm
wide) is seen in the metaphysis on the borderline with the cartilage in such cases. This is grade I osteochondritis. In
grade II osteochondritis this zone expands to 2-4 mm and spur-like outgrowths and inden-tation appear on the side
facing the epiphysis. The epiphyses of infants of the first months of life have a cartilaginous structure and are
therefore not demonstrated on the radiographs. Radiographs of the skeleton should be made no later than the first
three months of life because these changes may disappear with time. In the ab-sence of other signs of syphilis, the
diagnosis of congenital syphilis cannot be made on the basis of grade I osteochondritis alone, because these changes
may be encountered in the bones in other pathological conditions (hypervitaminosis D, hypotrophy, hypovita-
minosis, etc.). In a far advanced process (grade III osteochondri-tis) the zone of preliminary calcification disappears
as if washed away. A dark band of destruction, 2-5 mm wide, is found in the metaphysis. Replacement of bone by
granulation tissue may lead to intrametaphyseal fractures and the development of Parrot's pseudo-paralysis: in this
condition the infant presses the diseased upper limb to the trunk, flexes the diseased lower limb at the knee and hip
joints, avoids active movement and cries when passive movements are made (nerve conduction is preserved and
sensitivity and movements of the fingers and toes are therefore not impaired)
Syphilitic periostitis and osteoperiostitis in infants are mostly localized in the long tubular bones (which leads
to the formation of mildly pronounced organic, tender swellings on the bone surface) and less frequently in the flat
bones of the skull (which causes changes in its shape: frontal bossing of the skull, Olympian forehead). Periostitis
and osteoperiostitis are usually diffuse (exudative-proliferative process) but also may be gummatous. They become
most pronounced when osteochondritis disappears (at the age of 3 to 6 months and later). Syphilitic phalangitis or
dactylitis may sometimes occur, which is extremely characteristic of congenital syphilis. The proximal phalanges are
mainly involved; the pronounced character of the process diminishes in the direction of the distal phalanges. A
diffuse or gummatous inflammatory process causes cylindrical or fusiform thickening of the bones; the fingers and
toes swell and become bottle-shaped. Many phalanges are usually affected; in some cases the process is unilateral,
there are neither tender-ness nor fistulas, which distinguishes syphilitic dactylitis from tuberculous. Dactylitis may
be a monosymptom of congenital syphilis and usually occurs between the ages of 1 and 4-5 and even 6 years.
The eyes are the most frequently involved sensory organs. In con-genital syphilis of this age chorioretinitis
(involvement of the retina and vascular coat of the eye) is predominantly noted. A characteristic punctate 'salt-and-
' yellowish pigmentation forms on the periphery of the fundus of the eye, which, however, does not affect the
infant's vision, as a rule.
A diffuse innltrative process with subsequent sclerosis is found in many internal organs (liver, spleen, kidneys,
lungs, testes). The affection begins in intrauterine life, as a rule, in the form of round-cell infiltration and
proliferation of the connective tissue of the vascular wall. Later diffuse affection of the tissue of the organ occurs.
Entrance of T. pallidum into the body of the foetus through the umbilical vein leads to involvement of the liver in the
first place, which is enlarged and becomes firm (although it remains smooth). Ascites and jaundice (due to impaired
flow of bile and sclerosis of the liver) are rare, however. Affection of the liver causes weakness, a sallow skin,
anaemia and cachexia. A similar pro-cess underlies affection of the spleen which grows larger, in perisplenitis its
irregular and rough inferior margin is palpated. Intersti-tial diffuse pneumonia may develop which causes the infant's
death within the first days after birth. Such children rarely survive for several months; they suffer from dyspnoea and
their lips and the entire skin surface are cyanotic. Myocarditis, endocarditis and peri-carditis develop in involvement
of the cardiovascular system; the cardiac valves, arteries and veins may also be affected. Involvement of the kidneys
is manifested by nephrosonephritis. Protein casts, renal epithelium and red cells appear in the urine in such cases.
Oedema develops sometimes. The blood is often marked by anaemia, leucocytosis and neutrophilia. The thyroid,
pancreas, gonads, pituitary body and adrenals are often involved in the pathological process. The abnormalities
caused by their dysfunction are diag-nosed later on the basis of dystrophic changes and developmental defects in the
children or on autopsy. A relatively frequent occur-rence is involvement of the testes (specific orchitis) which
become firm, enlarged, but remain smooth; hydrocele develops sometimes.
Polyscleradenitis is found quite often: the inguinal, cubital, cervical and other lymph nodes become enlarged
and firm.
Involvement of the nervous system in congenital infantile syphilis may be manifested by meningitis)
meningoencephalitis and dropsy of the brain (hydrocephalus). Syphilitic meningitis may be acute or chronic. Nuchal
rigidity, restlessness and, in some cases, vomiting and short-term convulsions are encountered in these infants; they
also cry without cause. Pareses, paralyses and pupillary disorders may be attendant to the symptoms listed above in
me-ningoencephalitis. Hydrocephalus is marked by tenseness of the fontanelle, divarication of the sutures of the
skull, enlargement of the skull, vomiting, and bulging of the eyes. To confirm the spec-ific nature of involvement of
the nervous system, the results of examination of the cerebrospinal fluid (lumbar puncture) are taken into account.
The amount of protein and the cell count (lymphocytes) in the fluid are usually increased. The positive results of
serolog-ical reactions and IFT and TPI in the fluid are also given due consideration.
The course of congenital infantile syphilis may be conditionally divided into two periods: from birth to the age
of 3-4 months and from 3-4 months to one year. The first period is characterized by the prevalence of generalized
processes on the skin and mucous mem-branes, appearance of diffuse infiltration, syphilitic pemphigus, involvement
of the bone system (osteochondritis, periostitis, dactylitis), and by a variety of changes in the internal organs (in the
liver and spleen particularly) and nervous system. In the second period the syphilitic changes are much less
pronounced. The skin and mucosal lesions are mostly of a localized character (only occasional papular eruptions are
found). Periostitis and gummatous lesions predominate in the bones. Involvement of the internal organs and nervous
system is much rarer. The second period of infantile syphilis is a kind of interim between congenital syphilis of
infants and congenital syphilis of early childhood.

Congenital Syphilis of Early Childhood


This period of congenital syphilis occurs in children between the ages of 1 and 4 years, and is distinguished by
the scarcity of mani-festations. Moist and eroded papules or condylomata latum usually occur, or papular lesions and
flat condylomata form simultaneously in the anal region and on the genitals. Since flat condylomata are the prevalent
symptom of this period, the entire period is often called condylomatous. The papular eruption (if present) is
characterized by large lesions localized on the genitals, limbs, buttocks, less frequently on the face. The papules in
the inguinal, axillary and other (natural skin folds often undergo erosion. Hard, gummatous nodules may form on the
skin of some patients.
Affections of the mucous membranes of the mouth (eroded papules), larynx, vocal cords (making the voice
hoarse), and nose are sometimes found. Syphilitic rhinitis with purulent secretion develops in the last case. It is much
rarer in this period, however, than in congenital infantile syphilis.
In addition to the skin and mucosal lesions described above, affections of the bone system, mainly periostitis of
the long tubular bones (usually the tibia, less frequently the forearm bones), and involvement of the ocular coats are
encountered in this period. Involvement of the liver and spleen are less frequent; there is no gross enlargement or
firmness of these organs. In exceptional cases, however, particularly when no specific treatment is applied, cirrhosis
of the liver with atrophy of its tissue may develop, which makes the prognosis very grave.
Syphilitic meningitis, meningoencephalitis, mental retardation and other affections of the nervous system have
been described.
In many children of 1 to 4 years of age congenital syphilis follows a latent course with no clinical symptoms
and is diagnosed only on the basis of positive serological blood reactions, which, how-ever, may be negative in 15 to
20 per cent of children with the disease.

Late Congenital Syphilis


Late congenital syphilis is diagnosed in children at the age of 4 to 15 years and later. According to Pashkov, in
more than 70 per cent of cases the first clinical symptoms of late congenital syphilis are not preceded by
manifestations of early congenital syphilis. This suggested the possibility that congenital syphilis may follow a long-
term asymptomatic course similar to that of acquired syphilis.
In its course, late congenital syphilis resembles acquired tertiary syphilis because patients develop gummata or
gummatous infiltrations of the skin, mucous membranes and bones (gummatous periostitis and osteoperiostitis),
joints, internal organs and nervous system (gummatous meningitis). Tubercular syphilids may appear on the skin.
The endocrine glands are affected in some patients. The vessels of the brain or spinal cord may be involved in late
con-genital syphilis (which leads to pareses, paralyses and epileptiform seizures). Tabes dorsalis or juvenile general
paresis occurs in rare instances.
Besides the listed symptoms of late congenital syphilis, which are identical with tertiary syphilids of acquired
syphilis, there are groups of symptoms encountered only in late congenital syphilis. They are divided into three
groups. The first group consists of authentic, or unconditional, signs pathognomonic for late congenital syphilis. The
second group is composed of accessory signs, which suggest congenital syphilis when there are other signs
confirming the syphilitic infection (the results of serological blood tests, abnor-mal findings in the cerebrospinal
fluid, medical history and results of examination of the mother and child). The third group is formed of dystrophies
(stigmata), which are encountered in many chronic infectious diseases and are not specific for congenital syphilis.
The combination of several dystrophies in the presence of other evi-dence of the infection (e.g. positive
Wassermann's test in the blood of the child and mother), however, enables the physician to make the correct
diagnosis of the disease. Moreover, the presence of dystrophies helps in the diagnosis of parasyphilitic diseases.
Hutchinson's triad (interstitial keratitis, syphilitic labyrinthi-tis and Hutchinson's teeth) is an unconditional, or
authentic, sign of late congenital syphilis. Interstitial keratitis is characterized by redness and opacification of the
cornea, photophobia, lacrimation, and stable impairment of visual acuity. In most cases first one eye and some time
later the other are affected. The process lasts for a long time and resolution is extremely slow. Syphilitic keratitis is
often accompanied with iritis, iridocyclitis and chorioretinitis. Syph-ilitic labyrinthitis, or labyrinthine deafness,
occurring suddenly in a state of apparent well-being is characterized by diminished hearing, usually bilateral, and
tinnitus. The process is associated with infiltrative inflammation of the labyrinth and degeneration of the acoustic
nerve. If labyrinthitis occurs in a child whose speech is still undeveloped, there may be difficulties in pronouncing
sounds or the child may become a deaf-mute. Hutchinson's teeth are marked by dystrophy of the two permanent
upper median incisors which are shaped like a chisel and have a notched cutting edge. Before the eruption of the
permanent teeth, Hutchinson's teeth are identified on a radiograph. The complete Hutchinson's triad is a very rate
occurrence. Interstitial keratitis and Hutchinson's teeth are particularly frequent occurrences; they are diagnosed in
30 to 48 per cent of patients with late congenital syphilis.
The accessory signs of later congenital syphilis are: sabre shins, syphilitic chorioretinitis and gonitis,
Robinson-Fourniet's radial scars, frontal bossing of the skull, deformity of the nose (saddleback, opera-glass, 'goat'
nose), certain dystrophies of the teeth (kidney-shaped or 'tobacco-pouch' tooth), and some forms of neurosyphilis.
Sabre shins are marked by night pains and anterior bending of the tibia as a consequence of osteochondritis
suffered in infancy. In rickets the shins are bent laterally in the shape of a letter '0'. A similar deformity, however, is
encountered, although rarely, in Paget's disease and acquired syphilis. That is why sabre shins are accepted as an
accessory and not an authentic sign of late congeni-tal syphilis. The false sabre shin in acquired syphilis forms as the
result of osteoperiostitis suffered earlier, which had led to massive stratification of bones on the anterior surface of
the tibia. Sabre forearms are encountered far less frequently.
Chorioretinitis is characterized by small 'salt-and-pepper' pig-mented areas on the fundus of the eye. The
process involves the vascular coat of the eye and the optic disk. Syphilitic chorioretinitis is usually encountered in
early congenital syphilis (congenital infantile syphilis) but may also occur in later stages. Since chorioretinitis may
also be of tuberculous aetiology, it is considered not an authentic but an accessory sign of late congenital syphilis.
Syphilitic gonitis, or Clutton's synovitis, or joint (Clutton was the first to describe this condition) follows a chronic
course with impaired mobility and restriction of movements usually at the knees and, less frequently, at the elbows
or ankles. There is neither increase in local temperature nor tenderness. Chronic syphilitic hydrarthro-sis often occurs
with interstitial keratitis or Hutchinson's teeth, that is why some syphilologists regard syphilitic gonitis to be an
authentic sign of late congenital syphilis.
Serological tests, which are positive in almost all cases with gonitis, and the results of ex juvantibus therapy
help in making the diagnosis.
Robinson-Fourniet's scars are arranged radially around the cor-ners of the mouth, on the chin, and around the
lips, and form as the result of Hochsinger's diffuse papular infiltration suffered in infancy. In some cases these scars
may be rounded or pitted (like a thimble). Similar small scars may remain after a child had candidiasis, pyoderma or
burn, which sometimes leads to diagnostic errors.
Frontal bossing of the skull in congenital syphilis forms in the first months of life, whereas in rickets this
deformity becomes apparent by the second or third year of life. It results from the deposition of a diffusely ossifying
infiltrate in the frontal and parietal bones, leading to a bulging of the frontal eminences and the forma-tion of a
groove between them. Localized syphilitic hydrocephalus is a contributing factor. Some children with congenital
syphilis have a steeple skull.
Syphilitic rhinitis suffered in early infancy may sometimes cause deficient development of the bony or
cartilaginous part of the nose resulting in deformities of the nose, which are very typical of late congenital syphilis.
They include saddleback (protruding nostrils), opera-glass and 'goat' nose. In acquired syphilis a gummatous pro-
cess may also cause deformities of the nose.
Among the variety of dental dystrophies, the following are the accessory signs of later congenital syphilis. A
kidney-shaped (to-bacco-pouch) tooth (the four masticating cusps of the first molar are underdeveloped and occur as
thin projections; the tooth itself is small, the diameter of the masticating surface is less than the diameter of the neck,
which makes the tooth domed) and a 'pike' tooth (hypoplasia of the masticatory surface of the canine tooth, from
which a thin conical outgrowth resembling the tooth of a fish protrudes).
Affections of the nervous system in late congenital syphilis are diverse and are always attended with a positive
T. pallidum immobilization test, though Wassermann's reaction is negative in some cases. In some patients the
cerebrospinal fluid may be normal but abnormalities are found in it in most cases. Dementia (as a consequence of
syphilis of the cerebral vessels), Jacksonian epilepsy hemiparesis, hemiplegia, speech disorders, persistent headache
(s a manifestation of syphilitic hydrocephalus), and tabes dorsalis are among the forms of neurosyphilis. In children
with tabes dorsalis the knee reflexes are preserved for a long time and ataxia rarely occurs; various pupillary
disorders (anisocoria, the Argyll Robertson pupil, etc.) are mostly revealed. The juvenile form of general paresis may
occur (the memory weakens, speech is impaired, delirium and pupillary disorders develop followed by convulsions
and paralysis; the clinical picture is always attended with positive serological reactions and abnormalities in the
cerebrospinal fluid in which Wassermann's reaction is always sharply positive).
Among the numerous and diverse dystrophies encountered in children whose parents have various infectious
(e.g. tuberculosis) and non-infectious (e.g. alcoholism) diseases, we shall dwell here only on those which are most
common in late congenital syphilis, though they are not specific of syphilis alone. These dystrophies (stigmata) may
also form in the early periods (sometimes in infants); they are caused by metabolic disorders, endocrine dysfunction,
disorders of cardiovascular and nervous activity.
The following stigmata of late congenital syphilis are most significant:
(1) Avsitidiisky's sign consisting in thickening of the sternal end of the clavicle as the result of diffuse
hyperostosis. The right clavicle is affected most frequently. A radiograph verifies the clinical diagnosis;
(2) a high 'lancet-like', or 'gothic' hard palate;
(3) infantile little finger (Dubois-Gissard's sign); the little finger is short (Dubois sign) and the crease of the
distal joint on its dorsal surface is below the crease of the middle joint of the middle finger, while the little finger is
somewhat deformed and turned inward (Gissard's sign);
(4) axiphoidia, i.e. absence of the sternal xiphoid process (it should be borne in mind, however, that the process
may be turned inward, which creates the impression that it is absent);
(5) Carabelli's cusp, the presence of a fifth auxiliary cusp on the mastica-tory surface of the first upper molar;
(6) diastema, gaps between the upper incisors;
(7) hypeftrichosis in boys and girls and growth of hair on the forehead almost to the eyebrows;
(8) dystrophy of the skull bones, bossing of the frontal and parietal eminences but without a separating groove.
These dystrophies are of diagnostic importance if two or more of them are encountered in combination with
other signs (at least with one true authentic sign of Hutchinson's triad, or with more than one accessory sign) of
syphilis or with the findings of serological examination and medical history verifying the presence of the infection in
the children and their parents.
Serological blood tests which are positive in 70 to 80 per cent of patients with active manifestations, and
particularly the TPI, which is positive in almost 100 per cent of patients, help in the diagnosis of late congenital
syphilis.
Prevention of Congenital Syphilis
The timely detection and proper treatment of syphilis in women is the basis of prevention of congenital
syphilis. The role of examination of pregnant women is particularly important because they must be treated
promptly. According to authoritative instructions in force in the USSR, women's consultation centres are obliged to
reg-ister all women in pregnancy and subject them to clinical and serological examination for syphilis. Serological
examination is carried out twice, during the first and second periods of pregnancy. If the active or latent form of
syphilis is found in the pregnant woman, specific treatment (only with antibiotics) is conducted. If the wo-man had
been ill with syphilis earlier and had completed antisyph-ilitic treatment, specific treatment is nonetheless conducted,
which in this event is called preventive, for the purpose of ensuring the birth of a healthy offspring.
One or two weeks prior to childbirth, non-specific, false positive serological reactions may appear. Therefore,
if they are detected two weeks before childbirth, the expectant mother is not given specific treatment, but two weeks
later she is again examined and the child too. If the diagnosis of syphilis is confirmed, antisyphilitic treatment is
prescribed for mother and child. The newborns, whose mothers had been sick with syphilis and had received proper
treat-ment prior to and during pregnancy, are subject to thorough and comprehensive examination and subsequent
follow-up. The new-borns, whose mothers had been inadequately treated in the past and who for some reason had
not undergone preventive treatment during pregnancy, are subject to prophylactic treatment according to
programmes endorsed by the USSR Ministry of Public Health.
The Soviet system of dispensary care for the population (compulsory registration of all patients with syphilis,
the detection and treatment of all sources of infection and of persons who had been in contact with the patients, free-
of-charge highly skilled treatment, prophylactic examination of women in pregnancy and of the staff of children's
establishments, catering enterprises, etc.) contributed to the sharp reduction of contagious forms of congenital
syphilis. This is also promoted by the constantly maintained links of women's and children's consultation centres and
maternity homes with venereological dispensaries.
SEROLOGICAL REACTIONS AND TESTS IN SYPHILIS
Classical Serological Reactions
The standard, or classical, serological reactions for syphilis have become firmly established in the practice of
the public health system. They are used for making the diagnosis of the disease (as an auxiliary method of
examination in the presence of clinical manifestations of the disease and in the detection of T. pallidum in syphi-litic
lesions, and as the principal method in the latent form of syphilis). They also play an important role in appraising the
efficacy of antisyphilitic therapy: the physician appraises the quality of treatment by the given method from the rate
of negative reversal of the serological reactions. With other indices the reactions are also taken into account as a
criterion of complete cure. Finally, serological examination is carried out of individuals applying for employment at
children's and at catering establishments and at other places of this kind. The tests are also conducted regnlarly in
persons employed at these establishments. Serological examination is compulsory for donors and patients admitted
to somatic hospitals (neurological, psychiatric, for internal diseases, etc.).
The group of standard serological reactions includes Wassermann's reaction (with different antigens) and
Kahn's and Sachs-Vitebsky's (cytocholic) precipitin tests. Besides, Kolmer's test (Wassermann's reaction in cold) has
been given wider application recently. The Wassennann reaction and Kolmer's tests are carried out in two variants
(qualitative and quantitative methods).
The great variety of standard serological reactions is explained by the proved antigenic mosaic character of T.
pallidum and the resultant presence of the correspondingly numerous antibodies in the blood serum of a syphilitic
patient (reagins, complement-fixating protein and polysaccharide antibodies, agglutinins, immobilizins, antibodies
causing immune fluorescence, etc.). Different antibodies may predominate in each stage of syphilis and,
consequently, reactions with some antigens may already be positive while with others they may still remain negative.
Moreover, the relative specificity of the standard serological reactions obliges not just one but a complex of reactions
to be carried out so as to avoid diagnostic errors. It should be borne in mind that the entire complex may pro-duce a
false positive, non-specific result, in some cases. Positive serological reactions in blood are encountered in malaria,
typhus, relapsing fever, leprosy, brucellosis, pneumonia, scarlet fever, ma-lignant new growths, during menstruation,
two weeks prior to and two weeks after delivery, after taking alcohol, fatty foods and some drugs, in chronic
pyogenic processes, diseases of the liver, etc. The number of false positive non-specific results of standard serologi-
cal reactions increases with age. All this calls for extraordinary caution in interpreting the results of serological
examination in some cases and to regard these reactions as valuable auxiliary pro-cedures verifying the clinical
picture, the results of other laborato-ry tests (for T. pallidum, cerebrospinal fluid), and the results of confrontation.
Wassermann's reaction is known for its complexity; it is conduct-ed in a special serological laboratory. The
reaction is based on the complement-fixation phenomenon. Both specific antigens of T. pallidum and non-specific
antigens (extracts from organs of healthy animals, e.g. muscles of beef heart) are used. Fixation of complement by a
complex (lipoid antigen and reagin of the serum tested) occurs. A haemolytic system (sheep erythrocytes and
haemolytic serum) is used for indication of the complex formed. Wassermann's reaction is more sensitive when it is
made with the cardiolipin antigen. The modified Wassennann reaction in the cold (Kolmer's test) also proved to be
more sensitive. Kolmer's test is distinguished by doublephase temperature regimens (the first phaseat 18-20C for
30 minutes, the second phasein a refrigerator at +4 to 6C for 18-20 hours) under which fixation of complement
takes place. Kolmer's test demonstrates reagins in sera in which Wassermann's reaction produces a negative result
because of the low con-centration of the reagins in them. Most syphilologists, however, claim that Kolmer's test and
Wassermann's reaction with the cardiolipin antigen are more sensitive but less specific than the other modifi-cations
of Wassermann's reaction. That is why the physician should adopt a cautious approach to isolated positive results of
Kolmer's test and Wassermann's reaction with the cardiolipin antigen.
In sharply positive results of these tests they are repeated with diminishing doses of the patient's serum so as to
determine the reagin titre. The quantitative test for reagins helps in differentiating early and late latent syphilis and
makes it easier to appraise the efficacy of antisyphilitic therapy: a decrease in the reagin titre testifies to the efficacy
of treatment.
The precipitin reactions (Kahn's and Sachs-Vitebsky tests) are much simpler than Wassermann's reaction in
technical performance. Antigens of higher concentration and containing larger amounts of cholesterol are used. The
precipitate produced in these tests is clearly seen by the naked eye (sometimes a magnifying glass or an
agglutinoscope is used).
The degree of positivity of Wassermann's reaction, Kolmer's test and the precipitin reactions is designated as
follows: 4+ (sharp-ly positive), 3+ (positive), 2+ or 1+ (weakly positive), (doubt-ful), (negative). In quantitative
Wassermann's reaction or Kolmer's test that serum dilution with which the reaction still produces a sharply positive
result is recorded (e.g. 1:5, 1:10, 1:20, etc. 1:320).
It should again be emphasized that, according to numerous data, the results of Kahn's test coincide with those
of Wassermann's reaction in 93 to 96 per cent of cases (its non-specific results are recorded in 1.6 per cent of cases);
the results of the Sachs-Vitebsky test coincide with those of Wassermann's reaction in 95 to 99 per cent of cases (its
non-specific results are recorded in 1.3 per cent of cases). It has already been pointed out that Wassermann's reac-
tion, however, can also produce a non-specific result (particularly with the use of the cardiolipin antigen and when
the reaction is conducted in the cold).
T. pallidum Immobilization Test (TPI)
The TPI possesses the highest specificity among all the existing tests for syphilis. Its principal purpose consists
in the disclosure of false positive results of standard (classical) serological reactions. This is particularly important in
patients who reveal no clinical manifestations of active syphilis or have lesions of the internal organs or nervous
system which may be caused both by a syphilitic infection and by other infectious and non-infectious factors. It
would be hard to overestimate the role of the TPI in the recognition of false positive results of standard serological
reactions in pregnancy, when the fate (health) of the future child actually depends on the result of the treponema
immobilization test.
The essence of the TPI consists in the loss of mobility by T. pallidum in the presence of immobilizins of the
serum tested and active complement. The test is conducted under conditions of anaerobiosis. The techniques are
rather complicated and the reaction is therefore conducted in special laboratories. Immobilizins appear in the
patient's blood serum later than the other antibodies and the TPI therefore becomes positive later than the standard
serological reactions and the immunofluorescence test.
The test is considered negative if up to 20 per cent of treponemas are immobilized; weakly negative if 21 to 50
per cent, and positive if 51 to 100 per cent are immobilized. The percentage of treponema immobilization is
determined according to a special table. Some laboratories conduct the TPI today by a simplified procedure of
blended methods suggested by Ovchinnikov.
The result of the TPI is positive in patients with tropical treponematosis (pinta, bejel) and sometimes in
sarcoidosis, erythematosis, tuberculosis, cirrhosis of the liver, atherosclerosis and other diseases. With age, the
number of false positive results of the TPI grows.

Immunofluorescence Test (IFT)


The fluorescence test began to be used in syphilis serodiagnosis in the Soviet Union from the end of the sixties
(Bednova). It is distinguished from the standard serological reaction by higher sensitivity (that is why in some
patients it is positive even in the primary seronegative period of syphilis) with preservation of high specificity. Some
clinicians, however, claim that it is inferior to the TPI in specificity and cannot therefore be used instead of this test,
though its technique is much simpler. The reaction is performed in more than one modification: IFT-10 (which is
more sensitive), IFT-200 and IFT-abs (which are more specific).
The principle of the test lies in that the antigen from the pathogenic treponemas combines with the
corresponding antibodies of the blood serum of the syphilitic patient. The complex produced is, in turn, combined
with luminescent rabbit serum antibodies against human globulins. In cases with positive results the fluorescein
causes yellowish-green fluorescence of T. pallidum seen by means of a luminescent microscope. If the patient does
not have syphilis, no fluorescence is demonstrated. The degree of fluorescence is graded by plus signs as in the
standard serological reactions: 4+, 3+ and 2+ stand for a positive result, 1+ and absence of fluorescence ()
designate a negative result.
Serological Reactions in Various Forms of Syphilis
The IFT and Kolmer's test, as the most sensitive serological reac-tions, may be positive in the primary
seronegative period of syphilis. This, in accordance with the regulations adapted however, can-not be accepted as a
basis for the diagnosis of primary seropositive syphilis. Some patients produce an isolated positive result of Was-
sermann's reaction with a treponemal or cardiolipin antigen in this period.
At the end of the third or during the fourth weeks after the appearance of primary syphiloma, the standard
serological reactions be-come positive; the primary seropositive period of syphilis begins from this point. In the first
to second week of the primary seropositive period, the results of serological reactions become positive (1-2-3-4 +)
and the reagin titre grows (1:5, 1:10, 1:20, etc.). The IFT and Kolmer's test are already sharply positive in all
patients; the TPI is negative, as a rule or the immobilization percentage is very low. Primary seropositive syphilis is
also diagnosed in patients in whom the precipitin reactions (Kahn's and Sachs-Vitebsky's test) and Wassermann's
reaction with non-specific antigens have yielded even a single weakly positive result. With further development of
primary syphilis all serological reactions become sharply positive, the reagin titre grows (to 1:80,1:160), the IFT
remains sharply positive, but the TPI in most patients is still negative or weakly positive.
In secondary early syphilis the result of all standard serological reactions is sharply positive in almost 100 per
cent of cases, the reagin titre is highest (1:160, 1:240, 1:320), the IFT is 4+ and the TPI is positive in more than half
the patients, but treponema immobilization is low (40-60 per cent).
In secondary recurrent syphilis the standard serological reactions are positive in 96 to 98 per cent of cases (a
negative result may be found in monosymptomatic recurrent syphilis and in emaciated patients), the reagin titre
tends to decrease (1:60, 1:80), the TPI is positive in 85 to 90 per cent of patients, immobilization is pro-nounced (80-
90-100 per cent).
The tertiary syphilis is characterized by positive results of the standard serological reactions in 50 to 90 per
cent of cases and positive TPI in 98 to 100 per cent of cases (in high immobilization percentage).
As it is noted in the respective section, the diagnosis of late latent seropositive syphilis is made only on the
basis of positive blood serological reactions with compulsory verification by the TPI because mainly this test (and, to
a lesser extent, the IFT) allows true syphilitic (even 2+ or 3+) and false positive, non-syphilitic serological reactions
to be differentiated.
The various forms of syphilis of the nervous system and visceral organs differ in the frequency and pronounced
character of positive standard serological reactions. General paresis, for instance, is attended with sharply positive
standard serological reactions in 100 per cent of cases; in syphilis of the cerebral vessels, tabes dorsalis and syphilitic
affection of the cardiovascular system, these reactions are positive in only 40-50-60 per cent of cases. The TPI,
however, is sharply positive (90 to 100 per cent immobilization) almost in all of these patients.
In congenital syphilis the standard serological reactions are not determinative in the first two months of life
because they may be positive due to passive transmission of the reagins through the placenta. A positive result of the
TPI is also of no significance since the immobilizins transmitted passively from the mother to the foetus disappear
spontaneously within six months after birth. When infection occurs just before birth, the TPI will still be negative
(be-cause the immobilizins form later in this case), despite the presence of the syphilitic infection in the infant's
body. In congenital syphilis with active manifestations, the standard serological reactions may be negative in 1 per
cent of infants and in congenital syphilis of early childhood in 15 to 25 per cent of children (the TPI in the latter
case, however, is positive in 90 to 98 per cent of children).In late congenital syphilis even in the presence of active
manifesta-tions, the standard serological reactions are positive in only 70-80 per cent of patients examined, whereas
the TPI is positive almost in 100 per cent of cases.

EXAMINATION OF THE CEREBROSPINAL FLUID


Examination of the cerebrospinal fluid is widely practiced to determine syphilitic affection of the nervous
system, as a criterion of the efficacy of treatment (in individuals with abnormalities in the fluid prior to treatment),
and as one of the criteria of complete cure. In some cases the character of the abnormality in the fluid helps in the
diagnosis of the form of neurosyphilis.
Lumbar puncture is usually made with the patient seated. Fine (0.8-1.5 mm, better 0.4-0.5 mm in diameter)
long (10-12 cm) lumbar-puncture needles are used. The puncture is made at the point corresponding to the
intersection of two lines: a vertical line dropped along the spinous processes, and a horizontal line. The horizontal
line joins the iliac crests and passes between the spinous processes of the vertebrae. After puncture, 7-8 ml (but no
more than 10 ml) of fluid is collected into a test tube and then poured into two test tubes. One is sent to the clinical
laboratory (where a cell count is made, protein is determined and Nonne-Apelt's reaction and Pan-dy's test for
globulin are conducted) and the other to the serological laboratory (where Wassermann's reaction, Lange's colloidal
gold test or the paraffin, or mastic, reactions are conducted).
The minimum isolated abnormality in the cerebrospinal fluid is as follows: protein, beginning with 0.4 pro
mille; cell count, beginning with 8 cells per 1 mm 3, tests for globulin: Nonne-Apelt's reaction, beginning with 2+,
Pandy's test beginning with 3+, Lange's test more than two twos, and positive Wassermann's reaction. The fluid is
also considered to be abnormal when several of its values are at the upper limits of the norm (e.g. protein, 0.33 pro
mille, 6 cells per 1 mm3, Pandy's test + + , Lange's test 0011221000).
In the section of this textbook dealing with syphilis of the nervous system it is indicated in which form of
neurosyphilis and how often changes are found in the cerebrospinal fluid.
PRINCIPLES AND METHODS OF SYPHILIS THERAPY
Agents used in treating syphilitic patients are called specific, antisyphilitic. They are prescribed after the
diagnosis has been made or as a measure for preventing the development of the disease. They are also used in ex
juvantibus therapy as an aid in the diagnosis of doubtful cases (it is most frequently applied when syphilis of the
internal organs or nervous system is suspected). Once the diagnosis of syphilis has been made, treatment should be
started as soon as possible (within the first 24 hours in active forms). The earlier the treatment is begun, the better the
prognosis and the more effective the treatment. According to instructions issued by the USSR Ministry of Health and
endorsed on March 10, 1976, besides antisyphilitic agents producing a direct effect on T. pallidum (main-ly in the
period of active reproduction), non-specific agents are widely used to produce a general invigorating effect and help
the body to overcome the infection. Non-specific therapy is applied at the same time as the specific antisyphilitic
agents or in the intervals between courses of specific therapy. The later the treatment begins, the longer the causative
agent remains in the patient's body, the more non-specific therapy is substantiated.
Penicillin and its derivatives are now the principal agents used in treating syphilis. Penicillin is used as a
sodium or potassium salt. Its derivatives are longacting agents. These are ecmonovocillin, bicillin-l, bicillin-3 and
bicillin-5. A reserve antibiotic erythromycin is used in intolerance of penicillin and bicilliri. Individuals who cannot
be given injections of penicillin preparations or erythromycin may be given oral phenoxymethylpenicillin in doses
double those of water-soluble penicillin.
Water-soluble penicillin is given as a single intramuscular injec-tion every three hours around-the-clock in a
dose of 50 000 U (in body weight under 60 kg), 75 000 U (60-80 kg), and 100 000 U (over 80 kg). Ecmonovocillin
is injected once in 24 hours in a dose of 600 000 U (in body weight under 60 kg), 750 000 U (60 to 80 kg) and 900
000 U (body weight above 80 kg).
Bicillin-l and bicillin-5 are injected once in five days, bicillin-3 once in four days; the single bicillin doses also
depend on the patient's body weight. If the patient weighs less than 60 kg 1 200 000 U are injected, in a weight from
60 to 80 kg1 500 000 U, and when the weight is over 80 kg1 800 000 U. To avoid embolism (which may
happen when the drug is accidentally introduced into a blood vessel) the long-acting preparations are injected into
the muscle of the superolateral quadrant of the buttocks; the solution is intro-duced with a pause.
Erythromycin is given orally, 300 000 U (0.3 g) five times a day, every four hours with an interval at night, or
250 000 U (0.25 g) every six hours. In body weight less than 60 kg, the total dose is 30g, in 60 to 80kg it is 35g, and
in body weight of over 80kg40g.
The doses of penicillin, ecmonovocillin and bicillin for children are determined by their age according to the
existing instructions.
Bismuth salts and iodine preparations are used in addition to antibiotics in the treatment of syphilis patients.
Iodine preparations are prescribed in the interval between courses in late forms of syphilis (tertiary syphilis, syphilis
of the internal organs and neuro-syphilis, late latent syphilis, late congenital syphilis).
The group of bismuth preparations includes bioquinol, bismoverol and a water-soluble agent pentabismol.
Bioquinol (an 8 per cent qruinine iodobismuth suspension in neutral peach oil; it contains 25 per cent bismuth, 56 per
cent iodine and 19 per cent quinine; 0.02 g of metal bismuth is contained in 1.0 ml of the suspension). The daily dose
is 1 ml (2 ml is injected every other day or 3 ml once in three days). The total dose ranges from 40 to 50 ml.
Bismoverol (a 9.5 per cent suspension of basic bismuth salt of monobismotar-taric acid in neutralized peach oil; 1 ml
of the suspension contains 0.05 g of metal bismuth); 1.5 ml (a daily dose of 0.5 ml) is injected twice a week; 16 to 20
injections comprise the course. Pentabismol (1.0 ml of a water solution contains 0.01 g of metal bismuth); 2 ml are
injected every other day; the total dose is 40-50 ml. Before use the flask with bioquinol or bismoverol is heated in
40-45C water and the contents are shaken thoroughly till a homogeneous suspen-sion is produced. Bioquinol and
bismoverol, like the long-acting penicillin preparations, are injected with a pause into the muscles of the
superolateral quadrant of the buttocks. Pentabismol is injected without a pause.
Iodine preparations are prescribed as potassium iodide or sodium iodide solutions. One tablespoonful is given
on the first day, two tablespoonfuls on the second day and three tablespoonfuls three times a day on the third and
following days. The solution is taken in half a glass of milk after a meal. Solutions of gradually increas-ing
concentrations (from 2-3 to 7-8 per cent) are used. Treatment lasts 3 to 4 weeks.
Arsenicals (novarsenol, miarsenol, osarol) and mercurial prep-rations are practically not used today because
they are toxic and may cause severe complications. The existing instructions, however, contain a schedule according
to which these preparations are applied (general courses). This treatment is recommended for patients in-tolerant of
penicillin and erythromycin, when bismuth salts are con-traindicated, and in antibiotic-resistant cases. In practice
such si-tuations are very rare.
The instructions issued by the USSR Ministry of Public Health contain several schedules for antisyphilitic
treatment. Two methods are principally used, the chronically alternating and the continuous (treatment only with
antibiotics, without bismuth salts).
In the chronically alternating method treatment consists in re-peated courses of antibiotic therapy (penicillin,
ecmonovocillin, bicillin) with or without bismuth salts. The bismuth preparations may be prescribed simultaneously
with the antibiotics or after the course of antibiotic therapy is completed. Intervals are made between courses. These
intervals last two to three weeks in treatment with antibiotics alone, and four weeks in treatment with antibiotics and
bismuth salts. The number of courses in the chronically alternating method is determined by the stage of the disease.
The longer the duration of infection, the greater the number of courses prescribed. In treatment with penicillin
(ecmonovocillin or bicillin) alone three courses are prescribed in primary seronegative syph-ilis, five in primary
seropositive, six in secondary early, and eight courses in secondary recurrent, tertiary, late congenital, latent and
other late forms of syphilis.
In chronically alternating treatment with antibiotics and bismuth salts (simultaneous or successive) two courses
are prescribed in primary seronegative syphilis, four in primary seropositive, five in secondary early, and six in
secondary recurrent syphilis. In early latent syphilis six courses of simultaneous treatment with antibiot-ics and
bismuth preparations are prescribed. Late latent syphilis, tertiary late congenital syphilis, clinical or serological
recurrence, seroresistant and other forms of late syphilis are managed by six courses of antibiotic therapy and salts of
heavy metals, but by the successive method. It is advisable to change the bismuth prepara-tion in each course. The
total dose of the antibiotic for a course is determined according to the stage of the disease and the patient's body
weight. Patients with primary seronegative syphilis are prescribed 100 000 U of penicillin or its derivatives per 1 kg
of body weight; patients with primary seropositive or secondary early syphilis are given 120 000 U/kg, those with
secondary recurrent, tertiary syphilis and late forms of viscerosyphilis and neurosyphilis are given 14000 U/kg.
The continuous method of treatment with antibiotics alone is used in primary and secondary early syphilis and
only in some cases with secondary recurrent syphilis. In this method one third of the total dose of antibiotics is
injected in the form of water-soluble penicillin and two thirds in the form of a long-acting drug (bicil-lin). The dose
of antibiotic prescribed depends on the stage of the disease and on the patient's body weight. Thus, 300 000 U/kg of
the antibiotic is prescribed in primary seronegative syphilis, 480 000 U/kg in primary seropositive, 600 000 U/kg in
secondary early and 800 000 U/kg in secondary recurrent syphilis.
Women in pregnancy (with latent or active syphilis) as well as patients suffering from syphilis and active forms
of tuberculosis are not given bismuth preparations. Irrespective of the stage of the disease, women in pregnancy are
given 140 000 U/kg penicillin or its derivatives, the total dose should be no less than 8 400 000 U
Penicillin is injected in gradually increasing doses in late forms of syphilis and in pregnancy to prevent a
vigorous exacerbation reaction (in patients who had not been treated previously): 20 000
U are injected every three hours on day 1, 30 000 U every three hours on day 2, 40 000 U every three hours on
day 3, 50 000 U on day 4 (patients over 60 kg in weight are given 75 000 U every three hours on day 5, those with a
body weight of over 80 kg are given 100 000 U three times).
Infants under six months of age are treated only with penicillin (ecmonovocillin); six courses of treatment are
given. The dose for a course is 500 000 U/kg but no less than 2 000 000 U per course. Penicillin is injected round-
the-clock, 30 000 U are injected every four hours (the daily dose is 180 000 U). To avoid a vigorous exacer-bation
reaction, during the first course penicillin is injected in doses gradually increased every two or three days (2500-
5000-10 000-20 000 and further per 30 000 U). An interval of two weeks is made between the courses. Infants
between 6 and 12 months of age are also not given bismuth preparations. The course dose is 400 000 U/kg, but no
less than 3 000 000 U all in all. Penicillin is injected every four hours in a dose of 40 000 U (the daily dose is 240
000 U). The number of courses and the intervals between them are the same as for infants younger than six months.
These children may be given ecmonovocillin only beginning with the second course; 120 000 U are injected twice a
day. To avoid a vigorous exacerbation reaction, gradually increasing doses of penicillin are given during the first
course (5000 U every four hours, every two or three days the dose is increased by 5000 U till the full single dose is
reached). Children from 1 to 5 years of age are treated either with penicillin preparations alone (six courses) or with
penicillin and bismuth (six courses) if abnormalities are found in the cerebrospinal fluid, or the organs of vision are
affected, etc. The course dose is 300 000 U/kg, but no less than 3 000 000 U for children of 1 to 3 years of age and 4
000 000 U for those of 3 to 5 years of age. The single dose is 40 000 U (for children of 1 to 3 years of age) and 50
000 U (for children be-tween 3 and 5 years of age); injections are given every four hours. Ecmonovocillin is
prescribed beginning with the second course (150 000 U is injected twice a day). Bismuth preparations are given in
addition beginning with the fourth course. The intervals between the courses are prolonged to one month in this case.
Antisyphilitic therapy for children from 5 to 15 years of age is conducted either with penicillin or ecmonovocillin
alone (eight courses) or with pe-nicillin (ecmonovocillin) in combination with bismuth preparations (eight courses).
The bismuth preparations are given beginning with the fifth course. They are prescribed for children with abnormali-
ties in the cerebrospinal fluid or in involvement of the organs of vision. The dose of penicillin (ecmonovocillin) for
children 5 to 10 years of age is 300 000 U/kg but no less than 5 000 000 U, and 200 000 U/kg but no less than 6 000
000 U for children between the ages of 11 and 15. Children of 5 to 10 years of age are injected 60 000 U of penicillin
every four hours (round-the-clock). Those of 11 to 15 years of age receive 70 000 U every four hours. To prevent an
exacerbation reaction, the first course of penicillin is given in gradually increasing doses (10 000-20 000-40 000 U,
etc.; the dose is increased every day). Bismuth preparations are prescribed for children according to a special table.
The choice of the method of treatment is guided by the patient's age and general condition, the presence of a
concomitant disease which may be a contraindication for the use of some drug, drug tolerance, etc. In diseases of the
kidneys and liver, for instance, bismuth preparations are not prescribed.
As stated above, in addition to specific agents, non-specific measures are used in the treatment of syphilitic
patients. They include pyretotherapy (pyrogenal, prodigiosan), vitamins, ultraviolet irradiation, autohaemotherapy,
oxygen therapy, blood transfusions, injections of biogenic stimulators (aloe, placental and vitreous body extracts,
splenin, etc.), spa and balneotherapy. Pyretotherapy is used most widely. Pyrogenal is prescribed in an initial dose of
50-100 mpd (minimum pyrogenic dose), which is gradually increased to 200-300 mpd and more per injection
reaching to 1200-1500 mpd (depending on the body's reaction). The drug is injected once in two or three days; 8-12
injections per course. Prodigiosan is injected intramuscularly once in four days in doses of 25 to 125 g; 5 to 8
injections per course.
Preventive treatment is applied for individuals who had close intimate contact or everyday contact with a
patient with active form of syphilis. This is particularly important in regard to chil-dren whose parents have
contagious syphilitic lesions. If no more than two weeks have passed from the time of contact (sexual inter-course or
everyday contact), a single course of preventive treatment with penicillin, ecmonovocillin or bicillin is conducted;
100 000 U/kg are prescribed but no less than 6 000 000 U for a person with a body weight of less than 60 kg. In
cases when 2 weeks to 4 months have passed from the time of intimate contact with a patient suf-fering from
contagious or early latent syphilis, preventive treat-ment is applied according to the treatment schedule specified for
primary seronegative syphilis. Preventive therapy (a single course of penicillin therapy) is also prescribed for
patients with acute gonorrhoea, in whom the source of contamination has not been identified and who (for different
reasons) cannot be kept under longterm regular follow-up.
Complications may occur in the treatment of syphilitic patients Penicillin and its derivatives may cause allergic
reactions and toxic phenomena. The allergic reactions include anaphylactic shock, toxicodermia, acute oedema,
various eruptions; headache and haemopoietic disorders are the toxic phenomena. Penicillin drugs may impair the
activity of the intestinal flora, which is conducive to the development of dysbacteriosis (inhibition of E. coli activity
in particular). Dysbacteriosis, in turn, facilitates the development of candidiasis. Bismuth preparations may cause
complications in the kidneys (protein, casts, and 'bismuth cells' appear in the urine) and irritation of the oral mucosa
('bismuth margin', gingivitis, stomatitis). Rhinitis, iodine acne, and conjunctivitis may develop under the effect of
iodine drugs.
To prevent complications in antisyphilitic treatment, the patient must be examined carefully before therapy is
begun, detailed information must be gained on antibiotic tolerance in the past, and tolerance of the drugs during
treatment must be watched. The observance by the patient of the hygienic regimen (alternation of physical exertion
and rest, abstinence from spicy foods, alcohol, etc.), a fullvalue calory diet, vitamins and proper care of the teeth and
gums are also measures for preventing complications.

THE CRITERIA OP CURE AND INDICATIONS FOR TAKING


OFF THE RECORD COMPLETELY CURED PATIENTS

Patients who have completed antisyphilitic treatment are kept under dispensary care for two to ten years. The
term of control is determined by the period of syphilis in which treatment was begun. Individuals who completed
preventive treatment, for example, are taken off the record after a follow-up period of one year, those treated for
primary seronegative syphilis after two years, those treated for primary seropositive, secondary early, secondary
recur-rent, latent seropositive, tertiary active or tertiary latent, or congenital (all forms) after a period of five years,
and those treated for seroresistant, late visceral syphilis and late neurosyphilis are re-moved from the register after a
follow-up period of ten years.
During the control period, the patient visits the physician once in three or six months for medical examination
and serological tests (once in three months during the first year, once in six months dur-ing the second and third
years, and then once in 12 months). When the term of control ends the question of taking off the record is de-cided.
Taking the patient off the record, the physician proceeds from the premise that syphilis can undoubtedly be
cured. Evidence of this are cases of reinfection, the results of animal experiments, absence of any manifestations of
syphilis throughout the life in most patients who had been subject to proper treatment, and statistical data of
postmortem data of the last decades.
The absence of absolute criteria of complete cure, however, obliges us to weigh the sum total of criteria for
taking patients off the record.
These criteria are as follows:
(1) the stage of the disease in which treatment was begun (the earlier treatment was applied, the better the
prognosis);
(2) the time of the disappearance of the syphilids (epithelization or cicatrization of the chancre, disappearance
of the rose-coloured spots, resolution of papules and condylomata lata, etc.); a delay in the disappearance of
syphilids may be regarded as resistance to specific therapy and may lead to a less favourable prognosis;
(3) terms of negative reversal of the serological reaction (the earlier this occurs, the better the prognosis);
(4) the quality of treatment (in accordance with the instruction);
(5) the results of clinical and X-ray examination; the results of examination of the nervous system, internal
organs, fundus oculi and the cardiovascular system are particularly important;
(6) the results of laboratory (serological and cerebrospinal fluid) examination.
The reversal of positive TPI to negative in properly treated patients in the absence of syphilitic changes in the
internal organs and nervous system may testify to complete cure from syphilis. On the other hand, the TPI may
remain positive throughout life in individuals who began treatment late, which is no impediment to being taken off
the record if the results of all other types of examination are favourable.
According to the instruction, examination of the cerebrospinal fluid in taking off the record is compulsory for
individuals who were treated for primary seropositive, secondary, latent, tertiary, visceral, serorecurrent,
seroresistant, congenital syphilis and neuro-syphilis.
Persons who had begun treatment in childhood and children who had received preventive treatment must be
examined again (the nervous system and sensory organs) on reaching puberty.
SOFT CHANCRE (ULCUS MOLLE)
Soft chancre is caused by Ducrey's bacillus, or Haemophilus duc-reyi which in a smear of pus is arranged in a
chain or a 'school of fish'. Infection occurs during coitus when the bacilli enter in the injured skin and mucous
membranes of the genitals. Soft-chancre incidence has been practically eliminated in the Soviet Union, but
occasional cases may be encountered in port towns.
The bacillus is Gram-negative and stains readily with the main dyes (more intensively in the central part). The
bacillus is 1.5-2.0 m long and 0.4-0.5 m thick; it has rounded ends and is constricted in the middle.
Non-venereal transmission is extremely rare. Bacillus carriers are encountered. A female bacillus-carrier may
contaminate males. The disease leaves no immunity.
Clinical picture. A bright-red spot appears in two or three days at the site of entry of the bacilli, which then
develops into a tubercle and a small abscess. The last ruptures and an ulcer forms, which grows quite rapidly to a
diameter of 10-15 mm and larger. Many ulcers form, as a rule, which are in various developmental stages. They are
characterized by an irregular shape, eroded edges and soft consistency (hence the name 'soft chancre'). In distinction
from hard chancre, the soft chancre has an uneven floor covered with an abundant purulent secretion. Sharp
tenderness of the ulcers is another characteristic sign. In two or three months the ulcers heal, leaving a scar. Patients
with soft chancre may develop complications such as phimosis, paraphimosis, gangrene or phagedena, which are
described in the section dealing with complications of primary syphiloma. The inguinal lymph nodes are involved in
the process in 30 to 40 per cent of patients; inflammation and suppuration of the nodes occur, which rupture and
leave a scar on healing.
The clinical picture of ulcers in soft chancre and the regional lymphadenitis described above allows the
condition to be easily differentiated from syphilitic chancre and syphilitic regional scle-radenitis. The results of
examination for T. pallidum and Ducrey's bacillus and the findings of serological examination of the patient are very
important in the diagnosis. A mixed chancre (ulcus mix-tum) may develop in simultaneous infection with soft
chancre and syphilis. The clinical diagnosis is very difficult in such cases, though the soft chancre ulcers form much
earlier than the primary syphiloma or multiple hard chancres. Positive serological tests and eruptions of secondary
syphilis may appear in such cases only in four or five months. In view of this, all patients cured of soft chancre must
be kept under clinical and serological follow-up for no less than six months.
Treatment. Sulphanilamides and antibiotics are used with success in the treatment of soft chancre.
Sulphanilamides are taken in a dose of 3-4 g for eight to ten days. The use of penicillin or strepto mycin should be
restricted despite their high efficacy, because in cases with simultaneous infection with soft chancre and syphilis, the
antibiotics may render the picture of syphilis indistinct. A watery pulpy mass of sulphanilamide or sulphathiazole is
applied to the ulcers. Patients with a soft-chancre bubo are prescribed bed-rest and autohaemotherapy before the
nodes soften or small incisions are made in extensive and sharp softening after which an oil suspension containing
10 per cent Sulphanilamides is introduced into the cavity that forms.
GONORRHOEA
General Information
Gonorrhoea is a venereal disease caused by Neisser's gonococcus (Neisseria gonorrhoeae). In the process of
evolution, this causative agent became adapted as a parasite mainly on mucous membranes covered with columnar
epithelium. Unlike syphilis, the gonorrhoeal process is usually restricted to the urogenital organs and often affects
the rectum and sometimes the conjunctiva. Ilyin contends that only in exceptionally rare cases the infection acquires
a generalized character (gonococcal sepsis with bacterial metastasis to the joints and other organs).
Acute inflammation of the urethra in males and the urethra and the neck of the uterus in females is usually
attended with the discharge, of pus. This is precisely what provided grounds for Galen (2nd century, A. D.) to
suggest the term 'gonorrhoea' (Gk. gone seed, rhoia flow). Although this name gives an incorrect idea of the essence
of the disease, it has been fully preserved having ousted the synonyms 'blenorrhoea' and 'clap' used earlier. To be
sure, contagious diseases of males with discharge of pus from the urethra had been known long before Galen. In the
5th century . , for example, Hippocrates wrote about them and also reported on a white discharge from the
genitals of females. A discharge of almost a similar character, however, is attendant to inflammatory diseases of the
urogenital organs of various nature (infectious caused by micro-organisms and non-infectious). It was only the
discovery by the German scientist Neisser in 1879 of a peculiar micro-organism in the pus of a male with urethritis
that provided scientific grounds for regarding gonorrhoea an independent venereal disease, because this micro-or-
ganism unfailingly caused inflammation of the urogenital organs in humans.
Gonorrhoea today is one of the most prevalent bacterial infections. According to WHO experts, no less than
150 million people on Earth contract gonorrhoea every year. In some developed capitalist coun tries (such as USA,
France, Sweden and others) gonorrhoea incidence is second only to that of influenza among infectious diseases. In
the Soviet Union and other socialist countries, gonorrhoea incidence is much lower. The number of gonorrhoea
patients is nevertheless still considerable. The social significance of gonorrhoea as a venereal disease, is determined
not only by its high incidence and associated economical expenditure and difficulties, but by some consequences of
complicated gonorrhoea, sterility in the first place. After introduction of sulphanilamides, and especially
antibiotics, into gonorrhoea treatment, the frequency and severity of complications diminished noticeably, while the
clinical picture of gonor rhoea itself underwent marked changes (pathomorphosis). These changes consist in a certain
increase in the average duration of the incubation period, a mitigation of the inflammatory reaction and other signs
(Ilyin). Nevertheless, there are still fairly frequent cases of gonorrhoea with acute and severe complications, with
treatment-resisting consequences which not only venereologists, urologists and gynaecologists came up against, but
other medical specialists too (internists, surgeons, ophthalmologists and neurologists).
Aetiology. Gonorrhoea is caused by the Gramnegative diplococcus. The gonococci are lentil-shaped cocci
about 1.5 um long and 0.75 urn wide, arranged in pairs with their concave surfaces facing each other. They stain
readily by all aniline dyes. The gonococci change their morphological and tinctorial properties (the capacity for
assimilating a certain colour) under the effect of unfavourable factors to the point of becoming L-shaped. These L-
shaped cocci may appear not only in laboratory cultures, but directly in the human body when chemotherapeutic
agents are used or when the disease takes a chronic course.
The study of ultra-fine sections of the gonococcus with an electron microscope revealed that it consists of two
elongated cocci with a septum between them. On the outside it is completely covered with a scalloped six-layered
wall, which preserves the shape of the microorganism like framework. Immediately under the outer wall is the three-
layer cytoplasmic membrane tightly encompassing the cytoplasm. There are many grains, ribosomes, and a nuclear
vacuole contained in the cytoplasm.
Gonococci grow on artificial nutrient media in the presence of human protein at a body temperature of 37C.
In 1885 Bumm was the first to isolate gonococci in a pure culture with which he infect ed a healthy individual who
developed gonorrhoea of the urogenital organs. Some strains of the gonococci produce penicillinase, which explains
their relative resistance to penicillin and its derivatives.
Like other causative agents of venereal diseases, the gonococci are strictly human parasites. Outside the human
body they perish rapidly; heating above 56C, antiseptics, dessication, and direct solar rays destroy them. In pus the
gonococci retain their viability and pathogenicity only until the pathological substrate dries (from 30 minutes to 4-5
hours).
Under natural conditions no animal can be infected with gonococci. No success was achieved for many years
in creating an experimental model of gonorrhoea similar to the disease affecting humans. After numerous attempts
gonococcal sepsis was induced in mice by intraperitoneal injection of a great number of micro-organisms together
with mucin and dextrose. This experimental model proved usable for studying the efficacy of antigonorrhoeal agents
but was of very little help in elucidating the peculiarities of the pathogen-esis of the infection in man. It was only
very recently that a few scientists managed to reproduce gonorrhoeal urethritis in a male chimpanzee by means of a
gonococcal culture reinoculated several times; the male then infected a female chimpanzee by the sexual route.
Routes of infection transmission. Since gonococci are extremely unstable outside the human body, infection
usually occurs by the sexual route in direct contact of a healthy person with a sick indi vidual (or a seemingly healthy
gonococcal carrier). Adult males are practically always infected by the sexual route during a natural or perverted
sexual act. Gonococcal infection of the rectum and nasopharynx may develop in the latter case. In rare cases the eyes
of adults may become infected with gonococci through hands contaminated with secretions from the urogenital
organs; affection of the eyes in newborns results from infection from a sick mother during parturition. Non-venereal
infection occurs in rare cases through contaminated sponges, diapers or chamber-pots (usually in very young girls).
Infection from indirect contact is possible because gonococci remain viable and virulent for a short time while the
purulent exudate has still not dried up.
Pathogenesis. Congenital immunity to gonococcus does not exist in a human. A suffered disease also does not
produce true immunity and a person may therefore be repeatedly infected with gonorrhoea. Different antibodies
(complement-fixing, agglutinins, etc.) appear quite rapidly in the blood in gonorrhoea, but they do not prevent
complications or repeated infection. These antibodies evidently have no defence function but are 'witnesses' to an
existing or previously existing infection (Ilyin).
Phagocytic immunity also fails to develop in gonorrhoea. The purulent secretions of patients suffering from
acute gonorrhoea are usually found to contain many gonococci arranged in clusters both inside and outside the
polynuclear neutrophils. Phagocytosis, however, is incomplete. The gonococci do not die in the phagocytes, but, on
the contrary, they multiply. Such phagocytosis, therefore, does not protect the body from infection, but protects the
causative agents from the effect of specific immunity humoral factors (antibodies) and the natural resistance of the
body. The gonococci which had penetrated the polynuclears are carried by them to the adjacent organs and tissues.
Phagocytosis becomes completed (i.e. the causative agents are completely destroyed) if the gonococci had been
previously weakened by chemotherapeutic drugs, e.g. penicillin.
In males the causative agent of gonorrhoea gains entry through the urethra as a rule. The gonococci multiply
rapidly on the urethral mucosa, spread along it and penetrate between the epithelial cells into the connective-tissue
layer, urethral glands, and lacunae. They enter the posterior urethra gradually (but in some cases quite rapid ly) and
from there into the prostate along the numerous efferent ducts, causing inflammation of the gland. Much less
frequently the gonococci cause inflammation of the epididymis which they usually enter through the deferent duct
because of its antiperistaltic contractions. Lymphogenous spread of the causative agent is also possible. In females
the gonococci first enter the urethra, the neck of the uterus, and then may find their way to the uterus, uterine tubes
and the ovaries. Inflammation of the distal parts of the urogenital tract (urethra and neck) is sometimes attended with
general phenomena such as mild indisposition, subfebrile temperature, pain in the muscles and joints, which is
explained by the action of an endotoxin (gonococci do not produce a true toxin). Gonococci very rarely multiply in
the bloodstream causing the development of sepsis.
Gonococcal inflammation leads to degenerative processes in the epithelium of the urogenital organs. Erosions
may form on some areas. In chronic inflammation the columnar epithelium sometimes transforms to stratified
squamous epithelium with keratinization. A superficial diffuse infiltrate of lymphoid elements forms in the
submucosal layer of the connective tissue in the acute stage of the disease. With the development of the chronic
form, the infiltrate penetrates deeper, acquires a restricted focal character and may be replaced by cicatricial tissue.
GONORRHOEA IN MALES
Gonococcal infection in males usually occurs as affection of the urethra (urethritis). The disease develops after
a latent period of 5 to 7 days, on the average (from one day to two-three weeks and more). When the disease lasts
less than two months, it is referred to as fresh gonorrhoea. A disease lasting more than two months is conditionally
classified as chronic. Diseases of unknown duration marked by a torpid clinical picture with only a few symptoms of
inflammation are also related to chronic diseases. Fresh and chronic gonorrhoeal urethritis is always attended with
more or less pronounced objective signs of the disease, although subjective disorders may be absent. More and more
cases ate recently encountered however, in which males with gonococci in the urogenital organs have neither
subjective nor objective changes. This condition should be evaluated as latent gonorrhoea or a state of gonococcal
carriage (Ilyin).
The following forms of gonorrhoea of the urogenital organs in males are distinguished:
(1) fresh gonorrhoea:
(a) acute,
(b) sub-acute,
(c) torpid;
(2) chronic gonorrhoea;
(3) latent gonorrhoea.
All these forms may be attended with a variety of local and remote (metastatic, septic) complications.
Gonorrhoeal urethritis is characterized by a discharge of an inflammatory exudate from the urethra and
painful sensations of various intensity. In acute inflammation the lips of the external urethral opening are
considerably swollen and hyperaemic. In some cases they appear glassy and even slightly everted. An abundance of
yellowish-green or whitish-yellow pus flows continuously from the urethra. On palpation the cavernous body of the
penis is rather hard and tender. If the inflammatory process is restricted to the mucosa of the anterior urethra then,
when the patient urinates consequently into two glasses (the two-glass test) the urine in the first glass which has
washed out the pus accumulated in the urethra will be cloudy, whereas the urine in the second glass will be clear.
Patients with acute anterior gonorrhoeal urethritis usually complain of cutting pain at the beginning of
urination when the strong stream of urine stretches the inflamed urethral mucosa which is eroded in places. Painful
erection troubles some patients and in very severe inflammation (which rarely occurs) the penis is continuously in a
state of semierection. The purulent discharge in such cases may be sanguineous. Even if treatment is not applied the
acute inflammatory phenomena abate gradually in two or three weeks, the urethral discharge reduces, the subjective
disorders are alleviated and urethritis turns into the subacute and then into the chronic form. Acute total gonorrhoeal
urethritis develops when gonococci gain entrance from the anterior urethra to the posterior part of the urethra. In
such cases signs of posterior urethritis (urethrocystitis) appear in attendance to the symptoms of anterior urethritis.
The patients complain of frequent imperative urges to urinate at the end of which sharp pain is felt. In the two-glass
test urine in both glasses is cloudy because pus flows from the posterior urethra into the urinary bladder (total
pyuria). In some cases a few drops of blood are discharged from the urethra at the end of urination (terminal haema-
turia), which lends the appearance of meat washings to the urine in the second glass.
In many cases the gonorrhoeal infection is attended with mild inflammatory changes from the onset. In fresh
torpid anterior gonorrhoeal urethritis, hyperaemia and swelling of the lips of the urethral external opening can
hardly be seen or are absent. The urethral discharge is scanty and mucopurulent. Sometimes it is only noticeable in
the morning when the patient had not urinated at night, or only if it is pressed out of the urethra. The urine in the first
glass is clear and contains a few heavy, purulent threads and flakes precipitating to the bottom or it is slightly cloudy
(opales-cing). The subjective disorders in fresh torpid urethritis are mild: moderate pain at the beginning of urination
or a sensation of itching in the urethra. At times fresh torpid urethritis produces no subjec tive disorders, and the
disease is unnoticed by the patient. Extension of the inflammation to the posterior urethra in torpid urethritis is
likewise poor in symptoms. The diagnosis of posterior urethritis is only made when abnormal admixtures (purulent
threads and flakes) are found in the second glass of urine in the two-glass test.
Fresh subacute gonorrhoeal urethritis is encountered more frequently than the acute and torpid forms. In
objective and subjective signs it occupies an intermediate place between them.
Chronic gonorrhoeal urethritis resembles fresh torpid urethritis in its scanty discharge and mild subjective
disorders. Its course, however, may be interrupted by periods of exacerbation induced by alcohol, sexual excitation,
etc. Exacerbation of chronic gonorrhoeal urethritis resembles acute or subacute fresh urethritis in the clinical picture.
The correct diagnosis is helped by the medical history (a longer than two-month duration of the disease) and
urethroscopy (see textbooks on urology). Focal infiltrates (soft and hard), metaplastic mucosal areas and sometimes
strictures of the urethra are found in chronic gonorrhoea. Some type of complications, most frequently chronic
prostatitis, develop in males, as a rule.
Complications of gonorrhoeal urethritis in males. Acute gonorrhoeal urethritis, especially in males with a
long and narrow prepuce, may be complicated by inflammation of its inner fold and the glans penis
(balanoposthitis). and inflammatory phimosis which follow the same course as similar processes of non-gonococcal
origin. Abscess of the preputial gland is a rare local complication which is manifested by a moderately tender red
swelling of the frenulum of the glans penis. Sometimes the gonococci penetrate the paraurethral ducts where they are
less accessible to the effect of drugs and may therefore cause inefficacy of the treatment. Inflammation of the
paraurethral ducts is detected by thorough examination of the penis because they may open around the external
urethral orifice, on the glans penis or in the corona glandis or at any other site. The affected paraurethral duct is
palpated as a firm cord. When it is compressed a drop of pus is discharged from it. In some cases the inflamed
paraurethral duct has a punctate mildly infiltrated and hyperaemic opening on the urethral lips.
Littre's alveolar, tubular mucous glands and Morgagni's lacunae found in the urethra are practically always
affected by gonococci (littreitis and morgagnitis), Littreitis is marked by the appearance in the first portion of urine
of peculiar comma-shaped purulent threads which are impressions of the ducts of the urethral glands. In obstruc tion
of the excretory duct by the inflammatory infiltrate, small pseudoabscesses form. They are felt as tender thickenings,
slightly smaller than a pinhead when examined on a bouge or on the tube of the urethroscope. In some cases this
pseudoabscess grows to a considerable size. In timely and proper treatment, the inflammatory infiltrate usually
resolves, but in some cases purulent melting with the formation of a periurethral abscess occurs. When this abscess
is opened or ruptures spontaneously gonococci are not always identified in the escaping pus. It is possible that
pyogenic bacteria attendant to gonococci also contribute to the origin of this complication.
Infection of the excretory duct of Cowper's bulbourethral glands with gonococci (catarrhal or follicular
cowperitis) usually remains unnoticed by the patient. If no treatment is applied, acute inflam mation of the gland
itself results in the formation of an abscess attended with throbbing pain in the perineum and sometimes by painful
defaecation and frequent urination. Body temperature rises to 38C and even higher. Methods of objective
examination of the Cowper glands and other accessory sex glands in males are described in detail in textbooks of
urology.
Epididymitis, inflammation of the epididymis, was formerly encountered in gonorrhoea much more frequently
than now. Gonococci evidently penetrate the epididymis from the posterior urethra through the deferent duct, though
it is quite possible that the infectious agent is brought here in the blood or lymph. Inflammation of the duct itself
(deferentitis) develops in far from all cases of epididymitis, and it is therefore assumed that antiperistaltic contrac -
tions of the deferent duct in affection of the prostatic urethra, and especially the seminal colliculus, contribute to its
pathogenesis. Epididymitis is sometimes attended with effusion into the testicular coats (hydrocele, acute
periorchitis).
Gonorrhoeal epididymitis usually develops sharply. Pain in the affected epididymis (the testis itself is
unchanged in appearance, as a rule), hyperaemia of the corresponding half of the scrotum (the process is usually
unilateral), elevation of body temperature to 38-39C, indisposition, and headache appear simultaneously. The signs
of acute or subacute gonorrhoeal urethritis abate noticeably in this case. All the morbid phenomena increase for two
or three days and then subside gradually in three to four weeks. It is not always that the infiltrate in the tail of the
epididymis is completely resolved. It is often replaced by cicatricial tissue which compresses the lumen of the
epididymis. In bilateral inflammation azoo-spermia develops and causes sterility.
Inflammation of the prostate, prostatitis, is the most common complication of gonorrhoea in males. It is often
combined with inflammation of the seminal vesicle (vesiculitls). The glandular lobules of the prostate open into the
posterior urethra by means of numerous (30-40) excretory ducts. Infection of the pros tatic ducts usually
occurs in gonorrhoeal affection of the posterior urethra. When the inflammatory process is restricted to the excre-
tory ducts (catarrhal prostatitis) there are no subjective disturbances and the disease takes an asymptomatic course.
Spread of the affection to the lobules of the gland and the development of pseudoabscesses in them ( follicular
prostatitis) and, the more so, involvement of the interstitial tissue in the process (parenchymatous prostatitis) in an
acute disease leads to more or less pronounced systemic disorders combined with symptoms of acute posterior
urethritis. Patients complain of indisposition, pain in the perineum and above the pubis, increased body temperature
and dysuric disorders. Rectal examination reveals a tender, rather firm infiltration and enlargement of the affected
lobe or the entire prostate. Purulent melting of the infiltrate leads to the formation of a prostatic abscess. In such
cases the pathological phenomena are sharply intensified and acute retention of urine may develop. When modern
methods in the treatment of fresh gonorrhoea are applied in good time, acute prostatitis, the more so prostatic
abscesses, usually do not develop. They sometimes occur in patients who had not been treated for various reasons.
Chronic prostatitis, on the contrary, is very common in patients with protracted fresh or chronic gonorrhoea. It
may be consequent upon acute prostatitis or occur directly in the form of chronic inflammation (primary chronic
prostatitis). The pathogenesis of chronic prostatitis in gonorrhoea is complicated. Gonococci are detected
comparatively rarely in the secretions of the affected prostate even in untreated patients. The inflammatory process
in the prostate is usually not liquidated after complete destruction of the gonococci in the patient's body by means of
antibacterial agents. It is assumed that in such postgonorrhoeal diseases the inflammation is sustained by secondary
infection, neurodystrophic changes in the tissues, and phenomena of autoaggression (Ilyin and other authors).
Chronic prostatitis often has no subjective symptoms. Abnormalities are disclosed only on digital examination
and examination of the secretions (the leucocyte count in them is above 10-15 in the field of vision, the leucocytes
are gathered in clusters, the number of lipoid granules is reduced and crystallization of the secretion is disturbed). In
other cases chronic prostatitis is attended with symptoms of posterior urethritis: scanty urethral discharge, paraesthe-
sia, frequent urge to urinate and pathological inclusions in the urine. Prostatorrhoea develops sometimes: a few drops
of cloudy, fluid glandular secretion are freely discharged after urination or defaecation, this is a consequence of
impaired tonus of the smooth musculature of the excretory ducts.
Various neurotic conditions causing numerous and diverse complaints and sexual disorders are very common
in patients with chronic prostatitis, especially in long-term and unsuccessful treatment.

GONORRHOEA IN GIRLS
Young girls are infected with gonorrhoea by the non-venereal route, as a rule, due to inobservance of hygienic
rules in direct contact with sick adults or through objects contaminated with secretions containing gonococci. Older
girls (10-14 years of age) sometimes acquire the infection when a sexual act is attempted. Fresh, chronic and latent
gonorrhoea of girls is distinguished. Because of the age anatomical and physiological peculiarities, gonococci cause
inflammation of the vulva, vagina, urethra and often the rectum at the same time. Gonorrhoea in older girls is usually
similar to the infection in adult females.
Acute gonorrhoeal vulvovaginitis occurs five to seven days after infection and is characterized by bright-red
hyperaemia and swelling of the large pudendal lips, mucous membrane of the vestibule of the vagina, and the
perineum. Hyperaemia and swelling of the vaginal walls are found by vaginoscopy. Copious purulent or pyo-mucous
vaginal discharge often leads to maceration and inflammation of the skin of the perineum and inner surfaces of the
thighs. The patients are troubled by burning and itching in the region of the genitals and anus and painful urination in
cases of acute urethritis.
When the disease produces few symptoms from the very onset (torpid fresh gonorrhoea) and in cases of
chronic gonorrhoeal vulvovaginitis, the sick girl usually has no subjective disturbances whereas the parents' attention
is only drawn to the yellowish purulent spots on her underwear.
GONORRHOEAL PROCTITIS AND PHARYNGITIS
Gonococci enter the rectum either in the purulent secretions seeping from the pudendal cleft in women and
girls or during the perverted sexual act in male homosexuality. Only the distal segment of the rectum and the region
of the sphincter are affected; the gonococci find favourable conditions for existence here. Gonor rhoeal proctitis
rarely takes an acute course. A chronic course with very few symptoms is more common. Gonorrhoeal proctitis is en -
countered most frequently in girls and women and much less frequently in males.
Acute gonococcal proctitis is attended with painful defaecation or itching in the anus; admixtures of blood may
be found in the faeces when fissures and erosions form in the region of the sphincter. There is hyperaemia around the
anus, and pus accumulates in the folds. In chronic and fresh torpid forms of proctitis, patients have no complaints
and there are no external manifestations of the disease. Only proctoscopy reveals hyperaemia, swelling, a
mucopurulent discharge containing gonococci and, in some cases, erosions on the rectal mucosa.
Among other primary foci of gonococcal infection of extraurogen-ital localization, gonorrhoeal pharyngitis
and tonsillitis have been mentioned in recent years. They have been given particular attention by authors abroad
because pharyngeal (tonsillar) gonococcal infection is usually associated with infection during perverted (oro-
genital) sexual intercourse. Clinically it resembles catarrhal inflammation of banal origin, quite often with no
subjective disorders, and is discovered only by bacteriological examination. Unless they are treated, such
asymptomatic foci may become the cause of disseminated infection (gonococcal sepsis).
DISSEMINATED GONORRHOEAL INFECTION
In all probability, gonococci enter the blood stream in most cases of gonorrhoea, which is promoted by the
destruction of the mucous membrane of the urethra and cervical canal. In the blood, however, they perish
immediately under the effect of natural immunity factors. It is only in relatively rare instances that haematogenic
dissemination of gonococci occurs, when they multiply in the blood (gonococcaemia) and are carried to various
organs and tissues causing affection of the joints, endocardium, meninges, liver (abscesses, perihepatitis), skin, etc.
The dissemination of gonococci is facilitated by a long-term unrecognized disease, inadequate treatment,
menstruation and pregnancy, intercurrent diseases and toxicoses which diminish the body's resistance, and by injury
inflicted to the mucous membrane of the urethra or cervical canal during instrumental manipulations or sexual
excesses. Gonococcaemia, in recent years, has been somewhat more frequently encountered in females.
There are two main forms of disseminated gonorrhoeal infection. A rather rare occurrence is severe, at times
fulminant sepsis which clinically resembles septicaemia or septicopyaemia caused by other bacteria (staphylococcus,
meningococcus, etc.). It is precisely in this form that a severe general condition, marked fever, tachycar dia, a chill
and profuse sweating, as well as various skin eruptions (of the type of erythema nodosum, vesicular-haemorrhagic
and necrotic lesions) gain prominence. Polyarthritis with purulent effusion in the joints occurs simultaneously as a
rule.
A relatively mild course of disseminated gonococcal infection is encountered more frequently. The phenomena
of toxaemia are mild in such cases, the febrile reaction is moderate or short-term and affections of the joints
predominate in the clinical picture. This form is sometimes characterized as 'benign gonococcal sepsis'. It is probable
that in some instances the matter actually concerns transient bacteraemia with subsequent metastatic affection of the
joints in the form of mono- or oligoarthritis and skin eruptions, while in others a mild form of sepsis cavised by a
peculiar body reaction. The 'benign' character of this form of gonococcaemia is quite conditional, because it may
also be accompanied with endocarditis with involvement of the aortic and pulmonary valves, meningitis, abscess of
the liver, and other menacing complications.
The character and course of disseminated gonococcal infection are not determined by the state of the primary
focus, which is typical of all forms of sepsis. They are also not dependent on any especially virulent strains of the
causative agents. On the contrary, many scientists emphasize that typical causative agents, highly sensitive to
penicillin and other anti-gonorrhoeal agents, are isolated in disseminated gonococcal infections (and in gonorrhoeal
arthritis) as a rule.
Thus, gonorrhoeal arthritis is one of the manifestations of gonococcal sepsis or a consequence of short-term
bacteraemia, i.e. it is caused by the direct penetration of the causative agent into the periarticular tissues and joint
cavity. It is clinically similar to bacterial arthritis of other aetiology. The involved joint contains a purulent effusion
in which the causative agents can be found. The detection of gonococci in the synovial fluid indisputably confirms
the diagnosis of gonorrhoeal arthritis. The presence of gonococci in the urogenital focus and the
vesiculohaemorrhagic skin eruptions prompt the suspicion of the gonorrhoeal origin of the arthritis. Gon orrhoeal
arthritis easily responds to penicillin therapy. Treatment begun in time leads to full recovery and restoration of
function within a few days. If it is considerably delayed, however, destruction of the joint with subsequent ankylosis
may develop.
Reiter's syndrome (the urethro-oculosynovial syndrome) attended with persistent polyarthritis is encountered
more frequently than gonorrhoeal arthritis in males who had recovered from gonorrhoeal urethritis. This syndrome is
aetiologically unassociated with the gonococcus (see section dealing with non-gonorrhoeal urethri-tes in males).
DIAGNOSIS OF GONORRHOEA
The diagnosis of gonorrhoea (fresh, chronic or latent) may be established only when the causative agent has
been identified in the smears or cultures. Serological tests (the Bordet-Gengou phenomenon, test for gonococcal
antigen) as well as the skin-allergic test with the gonococcal vaccine are merely of auxiliary importance, but can
serve neither as proof of gonococcal infection in the given patient nor as a criterion of cure.
In acute fresh gonorrhoea the causative agents are usually detected easily by microscopy of smears stained in
parallel by the Gram-method (gonococci are Gram-negative) and methylene blue. In torpid and chronic gonorrhoea,
however, the results of bacterioscopy are less reliable. In a case with the corresponding medical history and clinical
picture, a negative result of one microscopic examination does not allow the diagnosis of gonorrhoea to be ruled out.
The reliability of bacterioscopy is somewhat increased by repeated examination, including that after provocation, i.e.
after artificial exacerbation of the inflammatory process. Combined provocation (biological by injecting the
gonococcal vaccine, alimentary, thermal, chemical and mechanical) is also necessary in ascertaining the cure.
Growth of cultures on artificial nutrient media in combination with microscopy practically double the number of
gonorrhoeal patients detected, particularly those with the chronic form, those who had been treated earlier, those
with involvement of the rectum, and others.
For the identification of gonococci by microscopy and in cultures, the pathological material is collected from
the urethra, prostate and seminal vesicles of males, from the urethra, cervical canal, rectum and, if indicated, from
the glands of the vestibule of the vagina of women, and from the vagina, urethra and rectum of girls.
Quite often other pathogenic micro-organisms are found together with gonococci in smears of the secretions,
which may be transmitted during sexual intercourse (urogenital trichomonads, Candida fungi, haemophilic vaginal
bacilli, etc.).
The mixed infection makes it difficult to detect the gonococci and is reflected in the clinical picture of
gonorrhoea: the duration of the incubation period increases and complications are more frequent. The gonococci
phagocytozed by the urogenital trichomonads do not perish within the protozoon but are, to a certain extent,
protected from the effect of the antigonorrhoeal agents. This explains some failure experienced in the treatment of
gonorrhoea. The penicillin-resistant trichomonads, haemophilic vaginal bacilli, Candida fungi and Chlamydia are
capable of sustaining inflammation of the urogenital organs after the death of the gonococci (postgonorrhoeal
diseases).
TREATMENT OF GONORRHOEA
Gonorrhoea is managed by means of antigonococcal agents (antibiotics and sulphanilamides), methods for
stimulating specific and non-specific immunity, as well as by different methods of local therapy the character of
which is determined by the localization and type of focal changes in the tissues and involved organs. Only antibiotic
therapy is applied in acute fresh uncomplicated gonorrhoea. A complex of measures is needed in protracted,
complicated and chronic forms.
The type and doses of antigonorrhoeal agents are established by periodically revised instructions in the
treatment and prophylaxi of gonorrhoea endorsed by the USSR Ministry of Public Health
(the latest instructions were issued in 1976). All Soviet physicians are obliged to follow these instructions
elaborated on the basis of the latest scientific medical data and the experience of clinical institutions.
The following agents possess an antigonococcal effect: benzyl-penicillin (in acute uncomplicated gonorrhoea
first 600 000 U is injected intramuscularly and then 300 000 U every four hours to a total dose of 3000 000 U);
ecmonovocillin-1 (600000 U injected every 12 hours to a total dose of 3 000 000 U); bicillin-1, bicillin-3 and
bicillin-5 (600 000 U every 24 hours to a total dose of 3 000 000 U); ampicillin (0.5 g taken orally every 4 hours to a
course dose of 3.0 g); tetracycline, chlortetracycline, oxytetracycline (0.3 g taken five times a day in the first two
days, and 0.2 g five times a day in the following days to a total dose of 5.0 g); erythromycin (400 000 U six times a
day for two days and then 400 000 U five times a day to a course dose of 8 800 000 U), oletetrin (on the first day 500
000 U taken orally in the beginning followed by 250 000 U taken three times, the remaining days 250 000 U is taken
four times a day; the total dose is 4 000 000 U); kanamycin (500 000 U injected intramuscularly every 12 hours to a
total dose of 3 000 000 U).
The course doses of antibiotics are doubled for patients with persistent, chronic and complicated gonorrhoea;
in gonococcal sepsis, gonorrhoeal arthritis and pelvioperitonitis 6 000 000-10 000 000 U of benzylpenicillin are
injected daily for 7 to 10 days depending on the patient's condition.
In antibiotic intolerance long-acting sulphanilamides, sulpha-monomethoxin and sulphadimethoxin, are
prescribed. For purposes of specific immunotherapy in chronic, complicated and torpid forms of gonorrhoea, in
cases unsuccessfully treated with antibiotics polyvalent gonococcal vaccine (gonovaccine) is injected, lacto- and
autohaemotherapy are applied as well as pyrogenal for increasing non-specific reactivity. The local methods of
treatment include irrigation of the urethra, instillation of silver nitrate (0.25 per cent) and Protargol, syn. silver
protein (1-2 per cent) solutions, introduction of metal bougies and tamponades, various types of physiotherapy
(paraffin and ozocerite application, diathermy, electrophore-sis, UHF therapy, massage, etc.).
Criteria of cure of gonorrhoea. The disappearance of the external signs of the disease after treatment does
not serve as evidence that the causative agents have perished, because they may persist for a long time in some of the
enclosed foci (latent gonorrhoea).
Full cure is determined 7 to 10 days after completion of treatment. For this purpose combined provocation is
carried out and then, 24, 48 and 72 hours later, smears are taken for bacteriological examination from the urethra and
urine and the prostate secretion in males, and smears from the urethra, cervical canal and other involved organs in
females. Whenever possible cultures are made simultaneously. In addition to bacteriological tests, urological (or
gynaecological) examination is carried out to reveal inflammatory foci in the urogenital organs. The provocation and
clinical examination are repeated in a month (females are examined during the next menstrual period). Individuals
who had suffered from gonorrhoea are kept under dispensary surveillance and laboratory control for two months
(females for at least two menstrual periods).
Individuals who had suffered from gonorrhoea are considered healthy and therefore taken off the record in
stable absence of gono-cocci in the smears and cultures, absence of inflammatory changes in the urethra and
accessory sexual glands in males (prostate, seminal vesicles, Cowper's glands), absence of pain or disturbances in the
menstrual cycle and obvious palpatory changes in the internal sexual organs in females.

TRICHOMONAL AFFECTIONS OF T UROGENITAL ORGANS


Trichomonal affection of the urogenital organs (trichomoniasis) is a widely spread disease caused by
Trichomonas vaginalis
Trichomonas vaginalis Donne (1836) is a unicellular micro-organism related to genus Protozoa, class
Flagellata. Its pear-shaped body reaches 13-18 m and more (up to 30-40 m) in length. The tricho-monads move
actively by means of movements of the flagella and an undulating membrane. The great plasticity of their bodies
allows them to produce pseudopodia and penetrate the intercellular spaces.
T. vaginalis is a parasite of humans and is adapted to living nowhere but in the urogenital organs. In the other
organs (intestine, stomach, etc.) and outside the human body the organisms perish rapidly because they do not form
any means of protection and are poorly resistant to unfavourable environmental factors. Dessication, heating to a
temperature above 45C, exposure to direct solar rays and changes in osmotic pressure have a particularly harmful
effect. Urogenital trichomonads therefore cannot be detected in the external environment, e.g. in open reservoirs, at
places where many people gather, in the sewages of public baths, etc. Under natural conditions they do not cause
diseases in animals in which other species of trichomonads parasitize. In experimental injection of pure cultures of T.
vaginalis subcutaneous abscesses, peritonitis, and vaginitis develop in laboratory animals.
Routes of infection spread. Infection is usually transmitted sexually. Rare cases of non-venereal contamination
occur in young girls, for the most part, through objects contaminated with the secretions of sick individuals (sponges,
wash-towels, etc.). This is possible because T. vaginalis remains viable for several hours in the clots of pus and
mucus (until the substrate dries up or is completely mixed in water). In the external environment, however,
trichomonads are even less resistant than gonococci; this is why non-venereal infection is much rarer in
trichomoniasis than in gonorrhoea.
Because of the common modes of spread, trichomonads often induce mixed infection with gonococci and some
other microorganisms.
Pathogenesis. Trichomonads parasitizing in the urogenital organs possess primary pathogenicity and are
capable of producing obvious or latent infection in all people. There is no congenital insusceptibility to them, but in
some persons infection results only in short-term (transitory) trichomonad carriage. In males, trichomonads may
parasitize in the urethra, paraurethral ducts, prepuce, epidi-dymis and accessory sexual glands. In females they
parasitize in the urethra, vestibular glands, the vagina, and the cervical canal. In rare cases the parasites penetrate
into the uterus and induce an ascending infection of the urinary tract (cystitis, pyelonephritis). In girls they cause
vulvovaginitis. Trichomonads cannot parasitize in the rectum and do not cause proctitis. Haematogenous dissemina -
tion does not occur. Thus, although several foci of affection usually develop in trichomonal infestation, they are all
restricted to the urogenital system. Despite such a character of the lesions, trichomonal infestation is attended with
the appearance of various antibodies in blood serum. The antibodies do not provide any noticeable protective effect,
however, and reinfection with trichomoniasis is therefore possible.
Clinical picture, course and complications. According to the course, fresh trichomoniasis (acute, subacute and
torpid), chronic (of a duration of over two months) trichomoniasis, and trichomonad carriage are distinguished. The
incubation period is 7 to 10 days, on the average, though in some causes it may be shorter (from 3 days) or longer
(up to a month and more). The clinical picture of trichomonal urethritis in males hardly differs from that of gonor-
rhoeal urethritis, but the disease and its complications (epididymitis, prostatitis, vesiculitis, etc.) most frequently
occur with moderately pronounced inflammatory changes or even with very few symptoms and with no subjective
disorders.
In acute trichomonal urethritis, extension of the inflammation to the posterior urethra produces the same
symptoms of urethrocystitis (frequent and imperative urges to urinate, pain at the end of urination, total pyuria,
terminal haematuria) as those in acute gonorrhoeal urethritis. Chronic trichomonal urethritis produces exa cerbations
at times resembling acute or subacute forms of the disease. Epithelial changes, infiltrative foci and cicatricial
strictures similar to those in gonorrhoea form in the urethral mucosa.
Symptoms of trichomonal infestation are usually more pronounced in females than in males. Signs of vaginitis
(hyperaemia and mild bleeding of the mucous membrane of the vagina and cervix uteri, thin, purulent, often foamy
secretions) prevail, as a rule, and may be combined with urethritis, endocervicitis, cervical erosions, and affections of
the vestibular glands. In acute vaginitis, the copious secretions induce burning and itching of the skin of the external
genitals. In the torpid and chronic forms there are often no subjective disorders. Girls develop acute vulvovaginitis
(or one with very few symptoms) with hyperaemia of the mucosa and secretions.
The diagnosis is based on identification of the causative agents in smears and/or in cultures. In males, it is not
the freely flowing secretions that are examined with the microscope, but scrapings or washings from the urethra, the
secretions of the sex glands (pros-tatic secretions, ejaculate) and the precipitate of freshly excreted urine. The
secretions from the cervical canal, urethra, and posterior vault of the vagina are examined in females; material
collected from the deep part of the vagina is examined in girls. Both native (in a hanging or crushed drop) and
stained specimens are examined with a microscope. Cultures are grown on special nutrient media. None of the
methods, however, ensures identification of the causative agents, and the examination must therefore be repeated
several times, combining microscopy with cultures whenever possible.
Treatment. In acute and uncomplicated cases an oral specific antitrichomonal agent Metronidazole (Trichopol,
Flagyl), or its derivates are prescribed; 0.5 g (two tablets) are given twice a day for 7 to 10 days. A complex of
measures (non-specific immunotherapy and local measures) is applied in complicated and chronic cases. In acute
gonorrhoeal-trichomonal infection antigonorrhoeal and antitrichomonal agents are prescribed simultaneously.
Chronic and protracted forms are first managed by immunotherapy (pyrogenal, lactotherapy) combined with
Metronidazole (Trichopol) medication, and only after that antigonorrhoeal antibiotics are prescribed.
Sexual intercourse is prohibited until the patient is completely cured and the causative agent has permanently
disappeared. Such individuals should be kept under dispensary surveillance while persons who had been in intimate
contact with them must be detected and treated, i.e. preventive measures, such as in gonorrhoea, are carried out.
Control tests (smears and cultures, urological and gynaecological examination) with alimentary and mechanical pro -
vocation are begun seven to ten days after the end of treatment and then repeated in one or two months for males,
and during three menstrual cycles for females. Girls are examined every month for three months. In the absence of
trichomonads in the smears and of inflammatory changes in the urogenital organs, the convalescents are taken off the
register.
NON-GONORRHOEAL (NON-SPECIFIC) URETHRITIS IN MALES
Inflammation of the male urethra caused by neither gonococci nor trichomonads but by other causes is called
non-gonococcal urethritis. Among the causes of these diverse conditions may be various factors of non-infectious
nature (mechanical and chemical injury of the urethral mucosa, allergy, new growths, etc.). Most frequently,
however, such urethritis is caused by microorganisms entering the urethra during intimate contact (urethritis of
venereal origin) or results from descending urogenic infection, causative agents brought from a prostate infected by
the haematogenous or lymphogenous route, etc. (non-venereal urethritis).
No doubts are harboured any longer that most cases of non-gonococcal urethritis are of venereal origin. Among
the causative agents of urethritis transmitted by the venereal route chlamydias are most prominent; 30 to 40 per cent
of cases with non-gonococcal urethritis are attributed to them. Haemophilic corynebacteria, II serotype-herpes
simplex virus, and fungi of Candida genus are isolated much less frequently. It has been suggested that definite
varieties of my-coplasmas contribute to the origin of some forms of urethritis.
The clinical picture of all types of non-gonococcal urethritis is characterized by the predominance of a torpid
course and very few symptoms as well as a tendency for a protracted course. Cases of an acute character resembling
the clinical picture of acute gonor-rhoeal urethritis are encountered. Non-gonococcal urethritis often produces
complications like prostatitis, vesiculitis and epididymitis, which differ from those of gonorrhoeal origin in a milder
course. The diagnosis of non-gonococcal urethritis is made when repeated bacterioscopy and bacterial tests for
gonococci and trichomonads are stably negative and no gonorrhoea or trichomoniasis is found in the sex partner.
Non-gonorrhoeal urethritis (with the exception of that of candidial origin) usually responds to treatment with tet-
racyclines (1.5 g given daily for 7 to 10 days). Persistent, protracted and complicated cases are managed by non-
specific immunotherapy and local treatment in accordance with the topical diagnosis.
Chlamydial urethritis deserves special attention because of its high incidence and the severity of the
complications attributed to it. Chlamydias are micro-organisms which take an intermediate place between rickettsia
and viruses. They include the causative agents of trachoma, venereal lymphogranuloma and some other human and
animal infections. The human urogenital organs are affected by Chlamydia oculogenitalis which closely resembles
the causative agent of trachoma. The morphology and biology of these organisms are described in textbooks of
microbiology.
On entering the urogenital organs during sexual intercourse, chlamydias multiply in the epithelial cells of
the urethra, rectum, vagina and neck of the uterus (and the conjunctiva in contamination of the eye) and cause an
inflammatory reaction. Urethritis developing in males after an incubation period of two or three weeks is usually
subacute or torpid. The chlamydial infection in females usually has no noticeable clinical manifestations, in rare
cases mild endocervicitis develops.
Chlamydias are inhibited by tetracycline, erythromycin and some of the water-soluble. sulphanilamides, but do
not respond to the effect of penicillin and its derivatives. It is believed that chlamydias cause postgonorrhoeal
urethral inflammation after penicillin therapy in cases with mixed infection as well as some of the articular
complications.
In 2 to 4 per cent of patients with chlamydial urethritis, the common complications on the part of the urogenital
organs are attended with the urethro-oculosynovial syndrome (Reiter's syndrome} This syndrome has a complex
pathogenesis in which hereditary pre^ disposition to articular lesions and altered body reactivity is of un doubted
significance. In many cases with Reiter's syndrome chlamydias were found in the scrapings from the urethra, the
synovial fluid of affected joints, and in the skin lesions. This syndrome is characterized by simultaneous or
consecutive involvement of the urogenital organs (urethritis, prostatitis, etc.), joints (poly- and oligoarthritides), and
eyes (conjunctivitis, uveitis, iritis). Skin eruptions of the type of keratoderma blennorrhagica, circinate balani-tis, etc.
may occur. The oral mucosa is sometimes involved. Reiter's syndrome follows a very torpid and persistent course at
times which does not respond to any drugs. In some cases spontaneous total recovery occurs after an attack of some
duration.
DISEASES OF THE SKIN OF THE PENIS
The skin of the penis, glans and inner surface of the prepuce may be the site of various cutaneous diseases,
namely, lichen ruber pla-nus, psoriasis, herpes, erythema exudativum, eczema, drug dermatitis, etc. The lesions may
occur with or without eruptions on other skin areas. Lesions associated with a venereal disease appear on the penis
particularly often. But lesions frequently form on the glans penis and inner surface of the prepuce, independently of
other skin or venereal diseases.
Inflammation of the glans penis (balanitis) is often combined with inflammation of the inner surface of the
prepuce (balanopos-thilis). A considerable amount of smegma accumulates in the pre-putial sac in untidy males,
particularly if the prepuce is narrow and long. The urine seeping into the preputial sac facilitates decay of the
smegma and activation of bacteria contained in it. The developing inflammation is characterized by itching, burning,
redness and swelling of the skin of the glans penis and prepuce. Erosions with a purulent discharge form on the
surface of the macerated skin. Superficial ulcerations may form if fusospirilary symbiosis develops in attendance.
The increasing swelling of the preputial skin leads to inflammatory phimosis. The retraction of the prepuce is at first
difficult and then becomes impossible. A copious amount of thin pus flows from the preputial sac.
Lymphangitis of the penis and inguinal lymphadenitis accompany inflammatory phimosis quite frequently. The
increasing circulatory disorders in individuals with reduced body resistance (chronic alcoholism, diabetes mellitus),
etc. are the occasional causes of gangrenous balanitis, and, at times, of fulminant gangrene of the prepuce extending
to the skin of the penis and scrotum. Marked systemic disorders and a febrile condition occur in such cases.
It is quite easy to reveal the cause of balanitis before phimosis develops. It should be borne in mind that
secondary balanoposthi-tis may develop in gonorrhoea, trichomoniasis, syphilis, Reiter's syndrome, pointed
condylomata, etc. With the development of phimosis, differential diagnosis becomes difficult, because syphi litic
scleradenitis cannot always be distinguished from common inguinal lymphadenitis on the basis of the external signs,
specific lymphangitis from non-specific lymphangitis, and inflammatory thickening palpated on the inner surface of
the prepuce through the skin from primary syphiloma. It is advisable to induce resolution of the phimosis to exactly
determine the origin of balanoposthitis. Drugs capable of masking the venereal infection must not be used in such
cases. The preputial sac must be freed from the smegma and pus by washing it out several times a day by means of
an urethral spout with warm solutions of potassium permanganate (1:6000), silver nitrate (1:1000), perhydrol (1
teaspoonful in a glass of water), 3 per cent boric acid or 1 per cent resorcinol. After irrigation, gauze strips with
dermatol (bismuth subgallate) or xeroform powder are inserted into the prepuce twice a day. If resolution of
phimosis is delayed and the swelling increases, operative incision or excision of the prepuce is resorted to
Chronic balanoposthitis with moderate hyperaemia and maceration of the skin and accumulation of a small
amount of whitish crumblike discharge may be a consequence of candidiasis which may oc cur both in venereal
contamination and by the non-venereal route.

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