Discussion
The collodion baby indicates a phenotype that precedes the underlying
disease entity. It is characterized by a yellow, shiny, tight
parchment-like membrane that covers the body. As collodion babies look
very much alike at birth, the outcome is difficult to predict. The
initial severity of phenotype and time to membrane shedding may be
indicative of the severity of the underlying condition. When the
membrane peels off, underlying skin disorders may usually be revealed.
It is considered that about 10% of all collodion babies heal
spontaneously within the first few weeks and develop normal underlying
skin.4,5 This condition is called “self-healing”
collodion baby or “lamellar exfoliation of the newborn.”
The benign and self-limited clinical course of self-healing collodion
baby distinguishes itself from lamellar ichthyosis. The cause of
self-healing collodion phenotype is not fully understood, and it is
considered as dynamic phenotype, which refers to the possible clinical
evolution of the disease over time.6 Thus, using the
term “self-improving collodion ichthyosis” as more appropriate is
suggested by some authors as many of the collodion baby patients showed
mild signs of ichthyosis when re-examined at 2–37 years of
age.7
Recently, mutations in the TGM1 gene, encoding epidermal
transglutaminase 1, which play an important role in lamellar ichthyosis
and nonbullous congenital ichthyosiform erythroderma, have been linked
to self-healing collodion phenotype.8 Also, mutations
inthe ALOX12B gene, encoding 12 (R) - lipoxygenase have been
described to be relevant for SHCB.7,8
The other diagnoses that need to be considered ii CB are the three types
of autosomal recessive congenital ichthyoses: non-bullous congenital
erythroderma, lamellar ichthyosis, and harlequin ichthyosis.2,3 Patients with lamellar ichthyosis have large,
dark, platelike scales involving the entire body surface, ectropion,
eclabium, and secondary nail deformity. These patients do not usually
improve with age. On the other hand, non-bullous congenital erythroderma
is characterized by generalized erythema and fine white scales, mild
ectropion, eclabium, hyperkeratotic palms, and soles. Harlequin
ichthyosis is the rarest and the most severe form, and it is associated
with respiratory and feeding difficulties, severe skin infections, and
lethal outcomes in early infancy.Other milder phenotypes include
ichthyosis vulgaris and X-linked recessive ichthyosis. Autosomal
dominant ichthyosis vulgaris usually manifest between 3 and 12 months of
age. X-linked ichthyosis is a genetic disorder caused by a mutation of
the enzyme steroid sulfatase involved in the metabolism of cholesterol
sulfate. It is presented at birth or early in infancy and affects males
exclusively. Rarely, Sjögren Larsson syndrome, Netherton syndrome,
Gaucher disease type 2, or some ectodermal dysplasias may be associated
with collodion membrane and should be considered if extracutaneous
symptoms are present.9
The management of collodion baby remains a challenge. Although the vast
majority of collodion babies are born at term, they require special
neonatal care as premature babies. Various complications may occur, such
as hypernatremic dehydration, hypothermia, skin infections, fissures,
conjunctivitis, sepsis, constrictive bands of the extremities resulting
in vascular compromise and edema.10,11 Placement of
the baby in a temperature-controlled, humidified incubator is essential
to reduce transepidermal water loss and prevent skin infections. Also,
most of the authors recommended maintaining the neonate in the incubator
for at least 4 weeks or until the membrane completely detaches.12,13
It is mandatory to practice all measures for the prevention of hospital
infections. The use of intravenous lines and blood sampling should be
very restrictive to avoid further skin damage. However, the prophylactic
use of antibiotics is not recommended. Ophthalmological management of
ectropion is essential for preventing conjunctivitis and
keratitis.13 The skincare includes applications of
bland emollients, as it was shown that they facilitated skin healing and
led to a relatively uncomplicated hospital course. 4
Recent advances in neonatal intensive care have improved the overall
prognosis of CB. The outcome depends on the initial assessment and
adequate multidisciplinary approach and treatment.