JDV_478.

fm Page 436 Tuesday, September 10, 2002 5:13 PM

JEADV (2002) 16, 436 437

EDITOR IAL

Collodion baby: whats new

Blackwell Science, Ltd

A Taeb,* C Labrze
Unit de Dermatologie Pdiatrique, Hpital Pellegrin Enfants, 33076 Bordeaux cedex, France. *Corresponding author, tel. +33 556 79 56 22;
fax +33 556 79 59 87; E-mail: alain.taieb@chou-bordeaux.fr

Van Gysel D, Lijnen RLP, Moekti S, de Laat PCJ,

Oranje AP. Collodion baby: a follow-up study of
17 cases. JEADV 2002; 16: 472475
The paper by Van Gysel et al. is the largest study reported at
a single institution (Queen Sophia Childrens Hospital,
Rotterdam, the Netherlands) for the condition named after
Hallopeau Bb collodion or collodion baby; this refers to a
collodion-like membrane that covers the baby at birth, which
is associated with major risks during the neonatal period,
including hypothermia, dehydration, infection and mechanical
compression leading to distal limb ischaemia. As confirmed in
the Rotterdam study, the overall prognosis has dramatically
improved over time with the development of neonatal intensive
care.1 This neonatal membrane is the most severe expression of a
congenital disorder of cornification of variable aetiology, including in the majority the most severe classic ichthyoses, which
are inherited as recessive traits, thus excluding the bullous
forms, at variance with the Rotterdam series (Table 1). The
diagnostic approach is summarized in Table 2. The transglutaminase assay allows us to refine the classification among the
non-associated congenital ichthyoses.2 The outcome is difficult
to predict, but the initial severity of phenotype and time to
membrane shedding may be indicators of the future severity of
the underlying ichthyosis.1 However, the resulting condition may
Table 1 Etiology of collodion baby syndrome
1. Common severe phenotypes (not associated with noncutaneous features)
AR congenital erythrodermic ichthyosis (50% of cases)
AR lamellar ichthyosis (10%)
2. Common milder phenotypes
Ichthyosis vulgaris type (mild form) (10%)
Recovery without sequaelae (10%)
3. Associated congenital ichthyoses
Trichothiodystrophy
Sjgren Larsson syndrome*
Netherton syndrome*
Gaucher disease type 2*
4. Miscellaneous conditions (collodion baby phenotype mentioned at
least once in the literature)*
Congenital hypothyroidism, Conradi syndrome, Dorfman Chanarin
syndrome, Ketoadipiaciduria, koraxitrachitic syndrome, ichthyosis
variegata, palmoplantar keratoderma with anogenital leukokeratosis
*True collodion membrane poorly documented.

436

Table 2 Major diagnostic procedures in collodion baby syndrome

1. Genetic anamnesis and family tree (consanguinity++)
2. Hair, eyebrows examination with polarized light (trichothiodystrophy,
Nethertons syndrome)
3. Search for lipid inclusions in leukocytes (Dorfman Chanarin syndrome,
Neonatal Gaucher disease)
4. Skin biopsy for transglutaminase assay, conventional and electron
microscopy. Keep frozen sample if possible for further studies
5. Blood sample for specialized biochemical and molecular studies
6. Others: neurosensory evaluation, bone X rays when needed

be quite mild (ichthyosis vulgaris type) or even difficult to detect

[this has been seen especially documented at the Unit de
Dermatologie Pdiatrique, Hpital Pellegrin Enfants (Bordeaux,
France) in patients diagnosed with trichothiodystrophy].
At birth, the clinical frontier of the most severe forms with
the so-called Harlequin fetus or kratome malin by French
authors, is now more blurred, as systemic retinoids have
allowed such patients to survive the neonatal period.3 In the
least severe forms, X-linked hypohidrotic ectodermal dysplasia
should be discussed if associated clinical dysmorphic features
are found, because overheating the baby may have tragic
consequences.
Why is the ichthyotic phenotype at its peak at birth? One
possible explanation is the absence of an airliquid interface in
utero that does not allow the epidermal differentiation
programme to progress to its complete development, which is
seen in normal human cultured epidermis maintained in
immersion. This may exacerbate the consequence of some
inherited epidermal differentiation anomalies.
The practical consequences for paediatric management
are manifold. A collodion baby is the equivalent of a highly
premature baby in terms of epidermal barrier, with a high
transepidermal water loss and a major risk of dehydration and
hypothermia.4 The baby must be admitted to the intensive care
unit to prevent this risk. Placement in a humidified incubator is
still considered as the most important decision, even though
susceptible to an increased risk of skin colonization with
harmful microbes. The incubator should be set at 90100%
hygrometry. The role of emollients is discussed in the Rotterdam
paper where they were found to increase the risk of infection.
At the Unit de Dermatologie Pdiatrique, Hpital Pellegrin
2002 European Academy of Dermatology and Venereology

JDV_478.fm Page 437 Tuesday, September 10, 2002 5:13 PM

Editorial 437

Enfants (Bordeaux, France) we bathe the child daily with

diluted chlorhexidine, followed by a rinse and then sterile petrolatum is used twice daily, which favours the shedding of
membranes and limits water loss. The discipline of hand
washing and the other measures to limit exposure to infections
routinely used in the intensive care unit are mandatory. The use
of intravenous lines and blood sampling should be severely
restricted to avoid further skin damage and the risk of systemic
infections. The weight of the child is the best clinical indicator
for the adaptation of nutriments and fluid intake. However, in
severe cases, in particular Nethertons syndrome (rarely clearly
documented at birth as a collodion baby), when hypernatraemic
dehydration is life threatening, an umbilical artery catheter may
be necessary.
The prophylactic use of antibiotics is not recommended. A
multisite assessment of the cutaneous microflora should be
done initially and monitored on a regular basis or when needed,
and cutaneous or systemic infections detected early and treated
appropriately. Ophthalmological management of ectropion is
important for the prevention of conjunctivitis and keratitis. The
management of pain should not be forgotten. A water bed is
useful and the prophylactic use of mild antalgics before daily
bathing and skin care is appropriate. Sedation with opioids may
be indicated when handling the child is very painful. The use of
retinoids may be considered when the shedding of the collodion
membrane is much delayed (after 3 weeks), because of the
infectious risk associated with the humidified milieu in
the incubator, and mostly because of the intrinsic severity of
the underlying ichthyosis. In that case, acitretin may be given at
0.5 0.75 mg/ kg per day.5
Eventually, one of the most challenging problems is to
communicate with the family of a first child born with this

syndrome. The long-term prognosis is difficult to address at

birth, and the compromised skin status of the child is difficult
to understand for the family. A very prudent approach is
mandatory to avoid further anxiety and a sense of guilt in the
parents, and to help them dealing with this distressing episode.
Coping develops quite naturally when the parents are allowed
to participate in the care of their baby. Prenatal diagnosis can
be discussed later in severe phenotypes.6,7

References
1 Larrgue M, Ottavy N, Bressieux JM, Lorette G. Bb collodion:
trente deux nouvelles observations. Ann Dermatol Venereol 1986;
113: 773 785.
2 Hohl D, Aeschlimann D, Huber M. In vitro and rapid in situ transglutaminase assays for congenital ichthyoses a comparative study.
J Invest Dermatol 1998; 110: 268 271.
3 Haftek M, Cambazard F, Dhouailly D et al. A longitudinal study of a
harlequin infant presenting clinically as non-bullous congenital
ichthyosiform erythroderma. Br J Dermatol 1996; 135: 448 453.
4 Buyse L, Graves C, Marks R et al. Collodion baby dehydration: the
danger of high transepidermal water loss. Br J Dermatol 1993; 129:
86 88.
5 Lacour M, Mehta-Nikhar B, Atherton DJ, Harper JI. An appraisal of
acitretin therapy in children with inherited disorders of keratinization.
Br J Dermatol 1996; 134: 1023 1029.
6 Pigg M, Gedde-Dahl T Jr, Cox DW et al. Haplotype association and
mutation analysis of the transglutaminase 1 gene for prenatal exclusion
of lamellar ichthyosis. Prenat Diagn 2000; 20: 132137.
7 Schorderet DF, Huber M, Laurini RN et al. Prenatal diagnosis of
lamellar ichthyosis by direct mutational analysis of the keratinocyte
transglutaminase gene. Prenat Diagn 1997; 17: 483 486.

Visit the EADV website at: www.eadv.org

2002 European Academy of Dermatology and Venereology JEADV (2002) 16, 436 437