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Veterinary Dermatology 2000, 11, 205210

Pigmentary disorders
Abstract There are several pigmentary disturbances which occur in the horse, some of which are primary
inherited conditions related to the breed, while others are acquired. All are related to the amount of melanin
present in the epidermis, dermis or hair and the activity of melanocytes in these areas may be independent of
each other. Within this section, a general discussion of leukoderma and leukotrichia is presented along with a
few specic conditions recognized as abnormalities of pigmentation.
Keywords: albinism, lethal, leukoderma, leukotrichia, reticulated, spotted.

LEUKODERMA AND LEUKOTRICHIA


General considerations
1 There are several skin diseases characterized by an
acquired loss of melanin pigment in the epidermis or
hair (Fig. 1).
2 It should be stressed that the melanocytes in the
epidermis and the melanocytes in the hair bulbs
frequently are aected independently.1 When the
melanocytes in the hair bulbs are aected and the
hairs lose their normal amount of melanin pigment,
the condition is referred to as leukotrichia.
3 When the melanocytes in the epidermis are aected
and the skin loses normal pigmentation, the condition is referred to as leukoderma (Fig. 2).
4 Another term that will be frequently encountered is
vitiligo, which also refers to an acquired loss of
melanin pigmentation in the skin. Although terminology is still changing, there is a tendency to use the
more general term, leukoderma, reserving vitiligo to
refer to a specic type of leukoderma in humans.
Aetiology and pathogenesis
1 There are several factors that may be involved
in the pathogenesis of leukoderma and leukotrichia, however, the exact aetiology and pathogenesis are unknown.
2 The following causes have been suspected as
triggers:
a. Trauma
b. Inammation
c. Heredity
d. Autonomic nervous system defect
e. Immunologic attack on melanocytes.
Clinical signs
1 It should be stressed again that the hairs and the
epidermis may be aected separately.
2 Leukotrichia without leukoderma is common.
3 Leukoderma, however, is usually accompanied by
leukotrichia.
4 There are several clinical syndromes (see below)
that occur in the horse involving leukotrichia and/or
leukoderma.
5 The prognosis varies. In some cases the loss of
# 2000 Blackwell Science Ltd

melanin pigmentation is permanent, while in others it


is temporary.
Histological ndings
1 There may be focal or complete loss of melanin
pigment from the epidermis (Fig. 3).
2 There may be a reduced number of melanocytes
within the epidermis and follicular epithelium (Fig.
4). This can be a dicult interpretation because the
melanocytes don't stain on H & E stained sections
when they have ceased melanin production.
3 Mild, focal pigmentary incontinence with dermal
melanophages or dermal melanocytes may be present.
4 Inammation is usually mild, and consists of a
perivascular mononuclear cell inltration in the
supercial dermis.
5 If interface dermatitis lesions are seen2 (apoptotic
cells, hydropic degeneration, lymphocytic lichenoid
inltrates) lupus erythematosus or a drug eruption
should be suspected.
Diagnosis
1 History and clinical signs are important are
important combined with compatible histological
ndings.
Clinical management
1 Currently, there are no eective treatments.
2 In certain instances, tattooing is utilized for
cosmetic purposes (the ethics involved in this type
of therapy should be considered).
RETICULATED LEUKOTRICHIA
General considerations
1 This is an idiopathic condition with very characteristic clinical features.
2 Quarter horses appear to be predisposed.
Aetiology and pathogenesis
1 Currently the aetiology is unknown.
2 Histopathologic evaluation of a few cases during the
crusted phase suggest the disease process is very similar
to, or represents a form of, erythema multiforme.
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A. A. Stannard

Figure 3. Leukoderma. The histological lesions may be subtle,


limited to focal loss of epidermal pigmentation. (H & E.
Magnication640.)

Figure 1. Leukoderma. Loss of pigment from lip and nasal area


(Case material: Stannard).

Figure 4. Leukoderma. A higher power photomicrograph at the


junction between normal and depigmenting skin. Note the mild
lymphocytic inammation. (H & E. Magnication6208.)
Figure 2. Leukoderma. Loss of pigment from the hair and from the
underlying skin; both leukoderma and leukotrichia (Case material:
Stannard).

3 This type of leukotrichia may be related to the


administration of various vaccines; there are special
implications to rhinopneumonitis vaccines, and a
proposed pathogenesis with this herpes virus vaccine
has been hypothesized3
4 In view of the predilection for Quarter horses,
hereditary factor(s) must be considered.
Clinical signs
1 Most horses develop the disease as yearlings.
Occasionally the disease develops in older horses.
2 The lesions develop on the dorsal midline from the
withers to the base of the tail.
3 The initial lesion consists of linear crusts arranged
in a characteristic cross-hatched pattern (Fig. 5).
4 When the crusts are shed, temporary alopecia occurs.
5 The new growth of hair in the aected areas is
white, i.e. leukotrichia (Fig. 6).
6 The crusts, alopecia and leukotrichia all develop in
the characteristic cross-hatched or reticulated pattern.
# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 205210

7 If the areas of leukotrichia are clipped it will be


noticed that the skin itself is normally pigmented, i.e.
leukotrichia without leukoderma.
8 The leukotrichia appears to be permanent.
Histological ndings
1 Early lesions of leukoderma and leukotrichia
are dierent.
2 There is a pronounced lichenoid interface dermatitis with apoptotic keratinocytes and satellitosis
mainly in the basal layer.
3 Dermal changes consist of supercial oedema and
pigmentary incontinence.
4 Intra- and subepidermal vesicles may be present.
Clinical management
1 There is no known eective treatment for the
condition.
2 Due to possible hereditary factors in the disease's
pathogenesis, consideration should be given to
removing aected animals from breeding programmes.

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Pigmentary disorders

207

Figure 5. Reticulated leukotrichia in an adult horse extending down


the back (Case material: Stannard).

Figure 6. Reticulated leukotrichia. Cross hatched appearance of the


white hairs in the same horse as Fig. 5 (Case material: Stannard).

SPOTTED LEUKOTRICHIA

JUVENILE ARABIAN LEUKODERMA

General considerations
1 As the name implies, spotted leukotrichia simply
refers to acquired spots of white hair (Figs 7 and 8).
2 It is highly unlikely that this is a single entity but
rather a clinical nding seen in several dierent
syndromes. Nevertheless, the subject deserves mention.
3 In general, leukotrichia appears to be more
common in horses with a hair coat colour with a
roaning tendency.

General considerations
1 Juvenile Arabian leukoderma is a characteristic
depigmenting disease in young Arabian horses. It
may also occur in young Quarter horses.
2 Juvenile Arabian leukoderma is the most common
form of leukoderma of horses in the absence of any
recognizable pre-existing or concurrent skin disease.
3 A similar pattern of leukoderma is occasionally
seen in mature Arabian horses and rarely in mature
horses of other breeds. The relationship between the
disease in young Arabian horses and mature horses
is unknown.
4 Clinical signs usually appear between one and two
years of age.
5 No sex predilection appears to exist.

Aetiology and pathogenesis


1 In some cases there is a history of prior skin lesions
or trauma. Micro-trauma could be involved in those
with no history of pre-existing lesions.
Clinical signs
1 One syndrome primarily involves Arabian horses.
2 Aected animals have multiple, sharply demarcated areas of leukotrichia.
3 Dorsal and posterior body surfaces are more often
aected.
4 Areas of leukotrichia range from 1 to 3 cm in
diameter.
5 Areas appear in sites with no known pre-existing
lesions. The lesions are not pruritic.
6 The natural course of the condition is variable.
Lesions may wax and wane, be permanent or resolve.
7 Co-existing leukoderma is variable. Those cases in
which a leukoderma is present have a much poorer
prognosis for repigmentation of the hair.
Histological ndings
1 The lesions are the same as described under
leukoderma and leukotrichia
Clinical management
1 There are anecdotal descriptions of horses with
spotted leukotrichia that have developed alopecia in
the aected areas and have subsequently responded
to systemic corticosteroid therapy.
2 Currently there is no known treatment.

Aetiology and pathogenesis


1 The predilection for the Arabian breed and the
occurrence of the disease in certain lines of Arabian
horses implicates hereditary factors.
2 The pathogenesis appears to involve an acquired
loss of functional melanocytes.
3 There is no evidence that an infectious agent or
nutritional factors are involved.
Clinical signs
1 The lesions are limited to the eyelids and periocular
skin, muzzle, nares, genitalia, anus, perineum and the
skin of the inguinal region and the undersurface of
the base of the tail (Fig. 9).
2 The only clinical sign of this disease is depigmentation. Initially, a few tiny macules of slate-grey skin
develop. These gradually increase in size and
continue to lose pigment over a period of one to
several months.
3 Erythema, scaling, crusting and pruritus are absent.
4 The syndrome will proceed in one of three dierent
patterns:
a. The aected areas remain permanently depigmented.
b. The aected areas spontaneously repigment and
the horse appears normal for the rest of its life.
# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 205210

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A. A. Stannard

Figure 7. Spotted leukotrichia. White


spots spread over the entire body (Case
material: White).

Figure 9. Juvenile Arabian leukoderma. Depigmentation of the


periocular region in a young Arab (Case material: Stannard).

6 Aside from the skin lesions, aected horses are


normal in all respects.
Histological ndings
1 These lesions are rarely biopsied and there were no
cases in Dr Stannard's material.

Figure 8. Spotted leukotrichia. Close-up of head of the same horse


as in Fig. 7 (Case material: White).

c. The degree of pigmentation waxes and wanes.


5 There are no reliable criteria available to
predict which of the above patterns any individual
will follow.
# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 205210

Diagnosis
1 The characteristic distribution pattern of leukoderma in young Arabian horses is diagnostic.
2 A major dierential diagnosis of leukoderma in a
muco-cutaneous distribution pattern in mature
horses is equine cutaneous lupus erythematosus.
Cutaneous lupus erythematosus lesions usually exhibit clinical evidence of inammation in addition to
the pigment loss.
3 The occurrence of somewhat similar lesions in
older Arabian horses, as well as mature horses of
other breeds, is a major unresolved problem.
Clinical management
1 There is no therapy.

226 DISC
Pigmentary disorders
2 Due to the probable inherited nature of the
condition, consideration should be given to excluding
aected animals from breeding programmes.
ALBINISM AND LETHAL WHITE FOAL
SYNDROMES
General considerations
1 Albinism refers to a congenital lack of melanin
pigment in the skin, hair and other normally
pigmented structures such as the uveal tract. Animals
with albinism have melanocytes.
2 This is an inherited disease.
3 Albinism may be classied as generalized or
localized and as complete or partial/incomplete.
4 The aected skin in albinism is characterized
microscopically as melanopenic rather than melanocytopenic. This distinguishes partial albinism
from piebaldism.
Aetiology and pathogenesis
1 These horses have a normal number of melanocytes in the skin. The defect is a biochemical one
in which the melanocytes lack the ability to
synthesize melanin.
Clinical signs
1 Most of the normal, inherited white markings that
occur on horses are localized forms of piebaldism.
2 Generalized and complete albino animals (oculocutaneous albinism) have white hair, white skin, pink
irides and usually exhibit photophobia.
3 Generalized albinism in the horse is inherited as
an autosomal dominant trait that is only viable in
the heterozygous state (Ww). These horses have
incomplete albinism as there is some colouration to
the iris (blue and white). Matings of such hetero-

209

zygous albino horses (Ww) produces a nonviable


embryo (WW) 25% of the time which is resorbed in
early gestation. This is one form of lethal white
foal syndrome.
4 A second form of lethal white foal syndrome is an
autosomal recessive disorder which occurs with
matings of overo paint horses. Lethal white foals
from such breedings are characterized by albinism
and congenital defects of the intestinal tract (colonic
atresia, ileocolonic aganglionosis)4 (Figs 10 and 11).
Histological ndings
1 There is a total absence of melanin but there are
melanocytes.
Clinical management
1 There is no treatment.
Editors' comment
The lethal white foal syndrome in the overo horse is
not associated with an albino phenotype. The heterozygote parents of these foals belong to a subset of
American paint horses with a distinctive piebald coat
colour pattern, known as frame overo. Lethality is the
result of homozygous expression of the gene associated with the frame overo colouration pattern. This
genetic defect, which aects development of neural
crest cells and intestinal ganglion cells, has been
identied as a missense mutation in the endothelin-B
receptor gene.5 In addition to the 40 frame overo
horses tested, several paint horses with tobiano
piebald markings also were identied as carriers.
REFERENCES
1. Pascoe, R.R.R., Knottenbelt, D.C. Manual of Equine
Dermatology. London: W.B. Saunders, 1999.

Figure 10. Lethal white foal


syndrome. Paint horse mare foal
(Case material: Carlson, University
of California, Davis).
# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 205210

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A. A. Stannard

Figure 11. Lethal white foal syndrome.


Close up of white foal shown in Fig.
10. Foal had congenital defects of the
intestinal tract on necropsy as well
(Case material: Carlson, University of
California, Davis).

2. Moriello, K.A., DeBoer, D.J., Semrad, S.D. Diseases


of the Skin. Philadelphia: W.B. Saunders, 1998: pp
513601.
3. Fadok, V.A. Update on four unusual equine
dermatoses. Veterinary Clinics of North America
Equine Practice 1995; 11(1): 1067.
4. Schneider, J.E., Leipold, H.W. Recessive lethal white in

the foals. Journal of Equine Medicine and Surgery 1978;


2: 47982.
5. Mettalinos, D.L., Bowling, A.T., Rine, J. A missense
mutation in the endothelin-B receptor gene is
associated with lethal white foal syndrome: an equine,
version of Hirschsprung disease. Mammalian Genome
1998; 9: 42631.

Zusammenfassung Einige Storungen der Pigmentbildung, die im Pferd auftreten, sind primare, vererbte,
rassenbedingte Syndrome, wahrend andere erworben werden. Alle stehen in Beziehung zum
Melaninvorkommen in der Epidermis, Dermis und in den Haaren, die Aktivitat von Melanozyten in diesen
Bereichen ist voneinander unabhangig.1 In diesem Kapitels werden allgemein Leukoderma und
Leukotrichosis zusammen mit einigen spezischen Syndromen, die als Pigmentstorungen betrachtet werden,
diskutiert. [Stannard A. A. Pigmentary disorders. (Storungen des Pigmentsystems.) Veterinary Dermatology
2000; 11: 205210.]

Resume Plusieurs troubles de la pigmentation sont decrits chez le cheval, certains primitifs et genetiques, lies a
une race, et d'autres secondaires. Toutes ces maladies sont dues a la quantite de melanine presente dans
l'epiderme, le derme ou le poil, et/ou a l'activite des melanocytes dans ces zones. Ce chapitre presente une
discussion generale sur les leucodermies et les leucotrichies, ainsi que quelques maladies associees a un trouble
de la pigmentation. [Stannard A. A. Pigmentary disorders. (Troubles de la pigmentation.) Veterinary
Dermatology 2000; 11: 205210.]

Resumen Existen diferentes alteraciones pigmentarias en el caballo, algunas de las cuales son primarias,
heredadas y asociadas a raza, mientras que otras son adquiridas. Todas estan relacionadas con la cantidad de
melanina presente en la epidermis, dermis y pelo, y la actividad de los melanocitos en estas areas puede ser
independiente entre ellas. 1 En esta seccion, se presenta una discusion general de la leucoderma y la leucotriquia,
junto con algunas anomal as espec cas reconocidas como anormalidades de la pigmentacion. [Stannard A. A.
Pigmentary disorders. (Alteraciones pigmentarias.) Veterinary Dermatology 2000; 11: 205210.]

# 2000 Blackwell Science Ltd, Veterinary Dermatology, 11, 205210

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