Collodion : A Rare Case Report

Dr Irman Permana, Sp.A1, Dr Ineu Nopita, Sp.A1, Dr Tatan Tandubela, Sp.A1, Luthfi
Ahmad I2, Hafiz Baihaqi2, Dandan Adi N2, Dyah Ayu L2, Metta Sari S2,
Mirellagreysalli K2, Ghea Gestivani S2, Galih Cahya P2,
1
Department of Pediatric Waled Hospital Cirebon 2Faculty Medicine Swadaya
Gunung Jati College Cirebon

Abstract : the term collodion baby is used for nrewborns in whom all the body
surface is covered by thick skin sheets, so called collodion membrane. Ichthyosis is
an infrequent clinical entity worldwide (1;300.000 births). When diagnosed in a
newborn, two forms can be identified : collodion baby and its most severe form.

Case report : We present the case of asian male infant who was born at 34 weeks gestation
in RSUD Waled Cirebon.physical examination the patient presented with a hard whitish
covering that involved the whole body, generalized edema, and eyelids with ectropion. The
ears had a dysmorphic appearance due to the keratotic coat, which retracted them and there
was fish like mouth called eclabium lips. Skin of the neck and trunk had the appearance of
fissures.

Conclusions : Collodion baby is a rare disease it is indispensable to have very clear

and precise information. Management of collodion baby need a multidisciplinary
approach, with family members, neonatologists, dermatologists and in some cases
other spesialist, all serving as active participants.

Keywords: collodion baby, ichthyosis, autosomal recessive

I. Introduction
About 270 cases of collodion baby have been reported since 1884. It is a
congenital disorder, occuring with an incidence of 1:300.000 live birth and both
gender are equally affected. Collodion baby is also known as collodion fetus.
Collodion baby is characterized by shiny, tight, cellphone-like membrane
stretched over the skin. The collodion membrane is a temporary condition, which
desquamates later on, and ultimately these children manifest sign of ichthyosis.
About 45% of collodion babies develop some complication due to skin barrier
function. It is associated with mortality rate of approximately 11%.1
The ichthyoses represent a large group of cutaneous disorders linked by
the common finding of abnormal epidermal differentiation. The word ichythyosis
 comes from the greek root ichthys, meaning fish, referring to the cutaneous
scaling that is characteristic of these disorders, which is said to resemble the scales
of a fish. Scaling can be localized or generalized and can be associated with a
fairly rare, it is important for the perinatologist and neonatologist to have some
familiarty with them, as the perinatal period and early infancy represent
particularly critical stages for patients with ichthyosis.2
In patient with ichthyosis, the barrier function of the skin is compromised
and has a decreased ability to protect against bacterial, chemical and mechanical
assault and to prevent transepidermal water loss. In infancy, the consequence of
this disrupted barrier can be particularly dangerous, at times life-threatening, with
increased metabolic demands due to increased epidermal turnover and evaporative
heat and water loss.2
II. Case Report

We present the case of aasian male infant who was born at 34 weeks
gestation in RSUD Waled Cirebon. Born by 22 years old multiparous mother. The
mother had prenatal care from the first trimester of pregnancybut do not check
ultrasonography examination. She was given prenatal vitamins and iron during
pregnancy by midwife and also received anti piretics for fever during labor.
During pregnancy the mother not have a history of severe illness and drinking
herbs. The patient was born by sectioseccariabecause placenta previa.
Apgarscoreof 5-6, respectively. He was given Vitamin K and oxytetrasiklin eye
ointment in the delivery room.

The patients birth weight was 2450 grams, length 46 cm and head
circumference 34 cm. Upon initial physical examination the patient presented with
a hard whitish covering that involved the whole body, generalized edema, and
eyelids with ectropion. The ears had a dysmorphic appearance due to the keratotic
coat, which retracted them and there was fish like mouth called eclabium lips.
Skin of the neck and trunk had the appearance of fissures. He was active and alert
and appropriately responsive during the examination. The physical examination
was unremarkable except for apparent inability to close the eyes completely and
limited range of motion in all joints due to the taut collodion membrane. Vital sign
Heart rate: 142 bpm, Respiratory Rate:40bpm, Temperature : 36,6oC
Figure.1
Figure.2
Figure.3
 Figure.4

III. Discussion
The term of collodion baby applies to newborns who appear to have an
extra layer of skin (known as a collodion membrane) that has a collodion-like
quality. It is a descriptive term, not a spesific diagnosis or disorder (as succh, it is
a syndrome). This exactly noted in our patient who had extra layer of transparent
skin.3
Collodion baby, sometimes referred to as collodion fetus is a common
presentation of several congenital ichtyoses, mostof which are inherited in an
autosomal reccessive manner, including lamellar ichthyosis (LI), congenital
ichthyosiform erythroderma (CIE) and self-healing collodion baby. It is a less
severe phenotype when compared with harlequin ichytosis (described below), yet
still has significant associated morbidity and mortalityCollodion babies are often
born prematurely and present at birth encased within a shiny, taut, cellophane-like
membrane. The tight skin around the eyes and mouth often leads to ectropion
(outturning of the eyelids) and eclabium (eversion of the lips), respectively. After
birth, the membrane begins to dry and fissure eventually leading to complete
shedding within the first several weeks of life, at which point the particular
clinical manifestations of the given underlying disease begin to develop. Other
disorders that can present as a collodion baby include trichothiodystrophy, neutral
lipid storage disease and Sjogren-Larsson syndrome, but these far less common.2
The major cause of collodion baby is not well known. However it has
been known to be inherited in an autosomal recessive fashion . Placental
insufficiency and post maturity have also been implicated in some forms of
collodion membrane formation. This could be due to the effects of DNA reppair
and transcription gene abnormalities in human pre-natal life. 2

3.1 Other forms of Ichthyoses.

Ichthyoses represent a heterogeneous group of skin disorders that are
characterized by a generalized scaling of the skin due to defective cornification
and desquamation. Children born with autosomal recessive congenital ichthyosis
(ARCI) often present at birth as collodion babies.4 Williams and Elias classified
ARCI into two major type using clinical, histological, biochemical, and molecular
genetic criteria: nonbullous congenital ichthyosis form erythroderma (NBCIE)
and lamelar ichthyosis (LI). The third type of autosomal recessive congenital
ichthyosis, Harlequin ichthyosis, is the most rare and severe form. The finding of
Van Gysel et al. Corresponded closely with those of Larregue et al. with the most
common outcome of collodion babies being erythrodermic autosomal recessie
lamellar ichthyosis in 3 of 17 children (19% of cases), and other forms in 2 of
children (12% of cases). In 4 of 17 children (10% cases), they found that skin
eventually developed normally, therein giving rise to a self-healing collodion
phenotype, as is the case with our patient.4
One important distinction between autosomal recessive congenital
ichtyhoses and other forms of ichthyoses is the age of onset of symptoms.
Whereas the tree types of autosomal recessive congenital ichthyoses typically
present with collodion membrane or ichthyosiform erythroderma at birht, other
 forms of ichthyoses such as ichtyosis vulagris and X Linked recessive ichthyosis
manifest after birth.4
Due to the impairment of the skin barrier function, collodion babies are at
risk for a number of complicationis, including hypernatremic dehydration,
hypothermia, skin infections, fissures, conjunctivitis, sepsis, dehydration and
constrictive bands of the extremities resulting in vascular compromise and
edema.2 The edema in patient described here was thought to be due to either
hypoproteinemia or mechanical compression by the collodion membrane. One
study showed that transepidermal water loss in collodion babies can be six to
seven times higher than that through normal skin. Skin impairment gives rise to
transepidermal water loss (TEWL), percutaneous infection, and toxicity. Accurate
appropriate adjustments of fluid intake. Measerement of TEWL with an
evaporimeter should be considered. Therefore, it is essential that collodion babies
be placed in a humidified incubator soon after birth to prevent hypernatremic
dehydration and hypothermia. The patient described here was managed
accordingly with the use of emollient and prophylatic antibiotics.2
3.2 Management.
Management of neonates with ichthyosis necessitates a multidisciplinary
approach, with family membes, nursing staff, neonatologist, demartologits and ,
in some cases other specialist, all serving as active participants. 1
Because many infants with ichthyosis are born prematurely, the primary
risk or complications are oftentimes those related to prematurity rather than skin;
therefore many of the medical concerns are similiar to those that neonatologists
manage on a routine basis. 1
The majority of therapies aimed at improving barrier function are fairly
simple and straightforward. The mainstays of management include providing
humidified, temperature controlled environment via an isolette, daily bathing with
only water or mild cleanser, and frequent, liberal applications of bland emollients
such as petrolatum based product. 1

Dehydration, Electrolyte imbalances, and increased metabolic demand. As

noted above, infants with ichthyosis should be maintained in a humidied
incubator. Urinary output, weight, and electrolytes should be monitored very
closely and managed with electrolyte repletion or intravenous hydration as
 necessary. A nutrition consult may be helpful in order to assist with determining
caloric requirements. In case in which obtaining peropheral intravenous acces
and/or transcutaneous monitoring is difficult de to markedly thickened skin, many
recommend placing umbilical line. 1

Infection. The use of prophylatic antibiotics in infant with ichthyosis remains

controversial. In case of more isolated cutaneous fissures and erosions, topical
antibiotics such as mupirocin can be used. 1

Skin Fragility, Risk of Systemic Absorption of Topical Medications, and

Pain Control. In many cases, neonates with ichthyosis have skin that is fragile
and more susceptible to injury from even minor trauma. In order to minimize this
risk, careful handling and avoidance of conventional adhesives is recommended.
Antibacterial soaps or washes such as chlorhexidine have the potential to cause
toxicity. For this reason, only bland emollients such as white petrolatum, vaseline
or aquaphorointment should be used and infants should be bathed in water only or
with very mild soaps or cleansers such as Dove or Cetaphil. 1

Ocular Complications. Infants must be evaluated for the prsence of ectropion,

which is commonly seen in cases of collodion baby. If present , opthamology
consult is recommended. Infants with ectropion are at risk for exposure keratitis,
so ensuring that the eyes are adequately lubricated is essential. Topical antibiotics
may be required for treatment of conjunctivitis or corneal abscess. 1

IV. Conclusion
Because this is a rare case and a rare disese it is dispensable to have very clear
information on the steps to follow and the complication that may arise. In order to
determine the etiologic cause for the collodion membrane, a protocol must be
established so that appropriate measures can be taken monts or years following
the shedding of the collodion membrane.
An incubator provides a humidified, neutral temperature environment. Other
supportive treatments such as intravenous fluid and tube feeding are often
necessary. The aim is to keep the skin soft and attempt to reduce scaling. The
collodion membrane should not be debrided (pulled off).
 In our experiene through this case study, we recommend careful attention to skin
care , minimal use of skin care products, and meticulous attention to preventing
infection when caring for an infant with collodion baby syndrome

REFERENCE

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Memorial Hospital, Raipur, Chattisgarh, India. 2015
2. Brittany G. Craiglow, MD. Ichthyosis in the Newborn.Department of Dermatology,
Yale University. New Haven, CT. NIH Public Acces.2012
3. Dr. (col) D. Y. Shrikhande, Dr Rajib Chattterje, dkk. Collodion Baby : A Rare Case
Report.IOSR Jurnal. Februari 2014
4. Michael Chung, Jaime Pittenger, Stuart Tobin, Andrew Chung, and Nirmala Desai.
Case Report Expedient Treatment of a Collodion Baby. Case Report in
Dermatological Medicine Volume 2011.