CASE REPORT

A New Born with Lamellar ichthyosis (Collodion Baby)

Zahid Gul, Gauhar Ali Khan, Fahad Liaqat and Kashif Muqarrab

ABSTRACT
Ichthyosis is an infrequent clinical entity worldwide with an incidence of 1:600,000 births. It can be one of the two types:
collodion baby and Harlequin fetus or malignant keratoma (most severe form). The clinical manifestations in either form
are thick and hard skin with deep splits. Affected babies are born in a collodion membrane, a shiny waxy outer layer to the
skin that is shed 10 - 14 days after birth, revealing the main symptom of the disease. The reported case is of a neonate,
born to primigravida mother at seven and a half month's gestation with a birth weight of 2160 grams and Apgar score of
6/10 and 8/10 at 1 and 5 minutes respectively. Conclusively, early diagnosis of this condition can help cope and prevent
serious morbidity or even mortality at time. These newborns should be monitored carefully in intensive care units by a
multi-disciplinary team.

Key Words: Lamellar ichthyosis. Newborn. Collodion baby.

INTRODUCTION during antenatal period. There was no significant past

The word ichthyosis is derived from the Greek
'ichthyosis' meaning 'Fish' due to the similarity in
appearance to fish-like scales.1 It is a very rare inherited
skin disorder, affecting around 1 in 600,000 live births.
The affected infant has a collodion type membrane, a
shiny waxy outer layer to the skin.2 The severity of
symptoms can vary enormously, from the mildest types
such as Ichthyosis vulgaris which may be mistaken for
normal dry skin up to life-threatening conditions such as
Harlequin ichthyosis. The most common type is
Ichthyosis vulgaris, accounting for more than 95% of
cases.3 The babies with such condition shed the skin
10 - 14 days after birth. With increasing age, the scaling
tends to be concentrated around joints in areas such as
the groin, the armpits, the inside of the elbow and the
neck. The scales often cover the skin and may resemble
fish scales.4 Clinically, the collodion babies may
encounter serious morbidities or even mortality due to
dehydration leading to electrolyte imbalance, hypo-
thermia due to altered perfusion, recurrent pneumonia or
respiratory problems and increased sepsis risk because
of severe skin damage.5
medical/surgical history.
General physical examination revealed mild anemia.
Obstetric examination revealed the baby was average
sized with cephalic presentation and normal CTG. She
was in the latent phase of labour. Labour augmentation
was done with oxytocin infusion. She had normal vaginal
delivery 2 hours after admission.
Patient delivered a male baby with birth weight of 2160
grams (below 5th Centile weight for age). Baby was
covered with white parchment. Apgar score was 6/10
and 8/10 at 1 and 5 minutes respectively. Baby had
features of rare congenital genetic disorder i.e. Lamellar
ichthyosis/Collodion baby (Figure 1 a,b,c,d). Baby was
kept in neonatal intensive care unit (NICU) for proper
management. A multi-disciplinary team was involved
and the diagnosis was confirmed after review by a
dermatologist. The baby was kept in incubator for
prevention of dehydration, intravenous IV line was
maintained and was started on intravenous (IV) fluid and

CASE REPORT
A 23 years old un-booked primigravida, housewife,
presented with history of seven and a half month’s
gestational amenorrhea and labour pains for 5 hours
before admission. She was married since 18 months.
There was no consanguinity. She had not received any
antenatal care and obstetric ultrasound was not done

Department of Pediatrics, Rehman Medical Institute, Peshawar.

Correspondence: Dr. Zahid Gul, Ashrafia Colony, Street
No. 09, Near Bilal Masjid, Peshawar.
E-mail: gulljee92@gmail.com
Figure 1 (a,b,c,d): Parchment like skin with cracks and peel, fish like mouth
Received: April 30, 2014; Accepted: November 21, 2014. and ectropion.

Journal of the College of Physicians and Surgeons Pakistan 2015, Vol. 25 (8): 621-622 621
Zahid Gul, Gauhar Ali Khan, Fahad Liaqat and Kashif Muqarrab
injectable antibiotic. The parents were counseled
regarding the disease and its future prognosis and
outcome.
DISCUSSION
Lamellar ichthyosis is known to be an autosomal
recessive genetic disorder; the carriers do not show any
sign or symptoms of the disorder.6 The term collodion
baby was first used in 1884 by Hallopeau.7
The term was used for newborns in whom the entire
body surface is covered with thick skin sheets, called
collodion membrane. The collodion membrane is the
result of an epidermal developmental dysfunction. It is
composed of thick skin sheets which resemble
translucent, tight parchment paper.8 The skin of a
collodion baby has a shiny film that looks like a layer of
vaseline. The taut membrane may impair respiration,
sucking and mechanical compression, leading to
problems like limb ischemia.4,5 There are fissures with
impairment of barrier to infection and water loss leading
to difficulties in thermal regulation and hypernatremic
dehydration.9 Neonatal morbidity and deaths may be
due to cutaneous infection (gram-positive and Candida
spp.), aspiration (squamous material) and hypothermia.8
The eyelids and mouth may have the appearance of
being forcibly open due to the tightness of the skin.
There can be associated eversion of the eyelids
(ectropion).
Treatment with a high-humidity environment and
application of non-occlusive lubricants may facilitate
shedding of membrane. Long baths, using bath oils or
application of lubricants before drying can prolong the
hydration and softening.9
The collodion membrane is peeled off or shed 2 - 4
weeks after birth, revealing the underlying skin disorder.
In addition to this, the membrane must be lubricated, to
achieve elasticity and desquamation, an adequate
hydration of the skin is the major components of
management. Suitable eye care and pain control should
be carried out for the collodion babies with ectropion.
Humidified incubators and water dressings followed by
622
emollient agents are the essentials of the management.
If there is a respiratory failure, ventilator support for the
collodion babies may be needed.10
In this case, a multi-disciplinary team was involved from
the day first to be ready for anticipated complications.
However, we did not document any electrolyte
imbalance. The C-reactive protein was normal and blood
culture showed no growth which ruled out sepsis. The
baby was kept in humidified incubator and was given IV
fluids, IV antibiotics, and hourly application of emollient
and frequent oiling of the skin with minimal and aseptic
handling. The patient was discharged on day 9 of
admission with advice to come for regular follow-up.
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Journal of the College of Physicians and Surgeons Pakistan 2015, Vol. 25 (8): 621-622
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