CASE REPORT

Niger J Paed 2013; 40 (3) : 307 –310

Obu HA

Collodion baby: A report of 4 cases

Adimora G

Chinawa JM

Obumneme-Anyim IN

Ndu I

Asinobi I

DOI:http://dx.doi.org/10.4314/njp.v40i3,23

Accepted: 28th December 2012

Abstract Introduction: The term

collodion baby refers to a clinical

entity noted in newborns who are

enmeshed by a translucent, corni-

fied substance like sheets of uni-

form texture so called (collodion

membrane) which gives the whole

body surface a varnished appear-

ance. Although, some other dis-

eases and conditions may lead to

collodion membrane formation, in

almost all the cases the cause is an

autosomal recessive ichthyosi-

form disease.

Case Presentation: The first three

cases are all from a consanguine-

ously married couple of Fulani

decent. The abnormal appearances

of the babies’ skin were all no-

ticed at birth. Parents are first

cousins. The last case is a female

born at GA of 40wks through

emergency caesarian section due

to poor progress of labor. She was

admitted into the Newborn with

abnormal skin.

These series are crucial so as to

enable the pediatrician have a high

index of suspicion of its existence

and to be equipped with the skills

to tackle the numerous complica-

tions that follow the disease.

They contribute to mortality and

morbidity among children in Nige-

ria and the exact etiology is un-

known. However genetic and envi-

ronmental factors among others

are commonly implicated.

This report is thus aimed at pre-

senting these abnormalities which

are rather rare and to highlight that

early intervention improves the

outcome in patients with these

conditions.

Conclusion: Early recognition of

this clinical entity and early insti-

tution of appropriate therapy can

definitely reduce morbidity and

mortality in neonates.

(

)

Chinawa JM

Obu HA, Adimora G

Obumneme-Anyim IN, Ndu I

Asinobi I

Department of Paediatrics

University of Nigeria, Enugu

Campus and University of Nigeria

Teaching Hospital,

Ituku - Ozalla, Enugu

PMB 01129,

Enugu, Enugu State, Nigeria.

Postal Code 400001

E-mail: josephat.chinawa@unn.edu.ng

Tel: +2348063981403

Key Words: Collodion Baby,

Neonates, Genetic Disorder

Introduction

the mode of inheritance as well as clinical and anatomic/

4,6

pathological data . Ichthyosis can be classified into

three groups: true ichthyosis, ichthyosiform states and

epidemolytic hyperkeratosis.

Collodion baby is a characteristic clinical entity which

may precede the development of one of a variety of ich-

thyoses or occur as an isolated and self-limiting condi-

tion. The first clinical description of collodion mem-

brane by Pérez in 1880 continues to be valid: “The

baby’s skin is replaced by a cornified substance of uni-

form t₁exture, which gives the body a varnished appear-

ance”. The most important clinical data concerning

collodion baby is the presence of disseminated or gener-

alized ichthyosiform genodermatosis characterized by

dry skin, scaling, generalized erythroderma and hyperk-

eratosis, reminiscent of fish scales. This type of derma-

There are several subtypes of each group. Among the

true ichthyoses are three subgroups as follows: auto-

somal dominant ichthyosis (ichthyosis vulgaris, ich-

thyosis simple, fish skin disease) X-linked recessive

ichthyosis (ichthyosis nigricans, ichthyosis of the male,

saurodermia) and autosomal recessive ichthyosis

(laminar ichthyosis,₇non bullous congenital ichthyosi-

form erythroderma). Ichthyosiform states are recog-

nized in the following syndromes: Sjogren-Larsson,

Conradi-Hunermann, Rudd, Comel, Tay, Refsum,

Netherton, Kid and Bid as well as erythrokeratoderma

variabilis of Mendes da Costa and progressive

2

-4

tosis is also known by the generic name of ichthyosis.

The tension exerted by the collodion membrane, distorts

the features of the face and fingers. Rarely, the shedding

of the membrane results in a normal integumen because

shedding of the membrane results in erythema of vary-

symmetric erythrokeratoderma.

5

ing intensity. The clinical types of ichthyosis depend on

Neonatal ichthyosis, in its most severe form, is known

3

08

as harlequin ichthyosis, harlequin fetus or maligna kera-

toma. Harlequin ichthyosis is also a keratinization disor-

der with extremely rare autosomal recessive hereditary

traits. ABCA 12 gene (adenosine triphosphate binding

cassette A 12), located at chromosome 2q33-q35,is rec-

ognized as the cause of lamellar ichthyosis and mutation

of this gene as being responsible for harlequin ich-

decent. The abnormal appearance of the baby‘s skin was

noticed at birth. Baby was delivered at home before

presenting in the hospital. The birth weight at presenta-

tion was 2.8kg.

On examination, there was ectropion and absence of

eyelashes and eye brows with an O-shaped mouth

(eclabium). The skin was semi transparent, and had a

parchment like feel with varying degrees of fissures at

groin, axilla and joint regions.

8

thyosis. The frequency of collodion baby is very low. It

is estimated that t₂h_,₄e_,₉re are 1:300,000 cases of newborns

in the worldwide.

We present these cases to highlight

The baby however died immediately of sepsis in the

hospital.

its rarity and need for presentation early to avert compli-

cations that follow it.

Fig 2a: Pictures of

Case 2: Note ectropion

on the forehead and

collodion membrane

on the body

Case Presentation

Case 1

B.H is a day old male baby, the first child of a consan-

guineously married couple of Fulani decent. The abnor-

mal appearance of the baby’s skin was noticed at birth.

Baby was delivered at home but mother presented im-

mediately to the hospital. On examination the baby

weighed 2.9kilogram.There was ectropion and the skin

was semi transparent and peeling. Systemic examination

was essentially unremarkable. Based on these findings,

she was managed as collodion baby. The baby was man-

aged in an incubator. The temperature, hydration and

electrolyte status were monitored. The baby was man-

aged with prophylactic antibiotics. Adequate nutrition

was provided by the use of expressed breast milk.

She received IV fluids and antibiotics. Baby did well

and was discharged.

Fig 2b: Note similar

ectropion on the limbs

Case 3

Fig 1a: Before Treatment

Baby M.H, is a two day old male neonate, the fifth child

in a polygamous family setting of Fulani descent. The

parents are first cousins. The mother did not receive

adequate antenatal care and the patient was delivered at

home by his paternal grandmother. The abnormal ap-

pearance of the baby was noticed at birth. The patient is

the third consecutive child of the mother with similar

presentation at birth.

On examination the baby weighed 2.5kilograms, with a

length of 48 cm and an occipito-frontal circumference of

3

4 cm. There was ectropion and absence of eyelashes

and eye brows with an O-shaped mouth (eclabium).

The skin was semi transparent, and had a parchment like

feel with varying degrees of fissures at groin, axilla and

joint regions. Systemic examination was essentially un-

remarkable.

Fig 1b: After Treatment

Note the disappearance of all

skin features

The baby was managed in an incubator. The tempera-

ture, hydration and electrolyte status were monitored.

Bland lubricants were applied to the skin to facilitate

desquamation. The baby was managed with antibiotics.

Adequate nutrition was provided by the use of expressed

breast milk.

The Collodion membrane peeled off within 19 days

revealing normal raw skin underneath. Patient was sub-

sequently discharged home in a fair condition. The first

of the preceding sibling of the index patient died of

severe sepsis due to late presentation while the second

Case 2

A.H is one day old female baby, the second consecutive

child of a consanguineously married couple of Fulani

3

09

was managed in a teaching hospital and improved

remarkably.

ichthyosis erythroderma (43%), lamellar ichthyosis

(19%), dominant ichthyosis vulgaris (12%) and normal

skin (25%). However, surveillance and genetic counsel-

4

Case 4

ing will continue during follow-up. Unfortunately, we

lost our patient to follow up. This is because their par-

ents are herdsmen and are always migrating.

NR is a female born at 40wks gestation through emer-

gency caesarian section due to prolonged labor. APGAR

score was 8/9 and birth weight 3.3kg.

On examination we found a baby with parchment like,

taut membrane covering the whole body. There was

bilateral ectropion, a flattened nose, an O shaped mouth

and dysmorphic fingernails. Other systems were grossly

normal.

A diagnosis of collodion baby was made. Full blood

count, Serum electrolyte urea and creatinine (SEUCr)

were normal. She was nursed in an incubator at high

humidity and was given IV fluids, antibiotics, multivita-

mins and genticine eye drops. Dermatologic and Oph-

thalmologic teams were invited to review, following

which she received keratolytics, moisturizer and sufratyl

gauze eye dressing. Blood culture results are negative.

The natural course of collodion membrane is intriguing.

For instance, approximately 75% of collodion baby will

go on to develop a type of autosomal recessive congeni-

tal ichthyosis, either lamell₁a₁r ichthyosis or congenital

ichthyosiform erythrodema).

Another 10% of cases the baby sheds this layer of skin

1

2

and has normal skin for the rest of its life. This is

known as self-healing collodion baby. This is akin to our

series. The remaining 15% of cases could stem from

1

variety of diseases involving keratinization disorders.

Known causes of collodion baby include ichthyosis vul-

1

3

garis and trichothiodystrophy. Less well documented

causes include Sjögren-Larsson syndrome, Netherton

syndrome, Gaucher disease type 2, congenital hypothy-

roidism, Conradi syndrome, Dorfman-Chanarin syn-

drome, ketoadipiaciduria, koraxitrachitic syndrome,

ichthyosis variegata and p₁a₃lmoplantar keratoderma with

anogenital leukokeratosis.

th

By the 10 day there was appearance of normal skin.

The patient was discharged on 11 day to Ophthalmol-

th

ogy and Dermatology Clinic. Unfortunately, she was

lost to follow up. It is important to note that none of

these patients presented with sepsis, electrolyte

imbalance or dehydration (safe the second who died of

sepsis) because of the early and vigorous management

which was given to them. These involve antibiotics,

fluid management and joint management with other

specialists.

There is a striking relationship between ichthyosis and

collodion membrane formation. Lamellar ichthyosis

may cause collodion baby. In these cases after the collo-

dion membrane peels, the skin is almost completely ery-

thematous and later on an almost generalized desquama-

tion is observed. Soon after, the entire body surface be-

comes covered by thick scales. Such₁a₄ thickening of the

stratum corneum is called ichthyosis.

Fig 3: Pictures of case 4

before treatment

Three of our series were associated with consanguinity,

the parents being first cousin. It has been noted with

interest that since many of these conditions have an

autosomal recessive inheritance pattern, they can be

1

3

associated with consanguinity. Frenk and his cohort, in

their study, noted that five spontaneously healing collo-

dion babies recorded in large Swiss kindred all had con-

sanguineous parents. Their distribution in the family

indicates autosomal recessive inheritance. A₁₅t birth they

had the typical features of collodion babies.

The major cause of collodion baby syndrome is not well

known. However it has been kn₆own to be inherited in an

1

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 repair and transcription gene

abnormalities in human pre-natal life. Trichothiodystro-

Discussion

The term collodion baby applies to newborns who

−6

phy (TTD), a rare (affected frequency of 10 ) recessive

appear to have an extra layer of skin (known as a collo-

dion membrane) that has a collodion-like quality. It is a

descriptive term, not a sp₀ecific diagnosis or disorder (as

disorder caused by mutations in genes involved in nu-

cleotide ex₁c₆ision repair (NER) pathway has also been

implicated. However our series were all born at term

with no placental abnormalities

The Collodion baby is at the risk of high losses of

transcutaneous fluid, risk of dehydration, hyponatremia

and skin infections (gram-positive and Candida spp.).

There is also the risk of pneumonia secondary to asp₃_,i₄r_,a₁-₇

tion of desquamated material in the amniotic fluid.

1

such, it is a syndrome). This is exactly noted in our

patients who had extra layers of transparent skin.

When the Colloidion membrane was shed, the underly-

ing skin appeared normal however a skin biopsy would

be performed by the dermatologists to determine the

specific type. The evolution reported in some studies

included various types of ichthyosis: congenital

3

10

our first and third cases were managed in a humidified

incubator and were given antibiotics but the second se-

ries died of sepsis due to late presentation. Taïbeb re-

ported that one of the most important decisions is plac-

ing the babies in₈ incubators with humidifiers that vary

ment of signs of sepsis and, meticulous fluid and elec-

trolyte balance. Constriction bands if present should be

divided.

In the third case, shedding of the Collodion membrane

occurred by the second week of hospital stay and no

keratolytic agents were used.

1

from 90-100%. Fluid and broad spectrum antibiotics

were also used. We did not document any electrolyte

imbalance.

4

, 19

affected babies were

In the study by other workers

Conclusion

treated with emollients as prophylaxis. While one report

documented systemic infections, the other study did not.

In our two series, we used bland emollients for lubrica-

tion and no infection was documented.

Because this is a rare disease it is indispensable to have

very clear and precise information on the steps to follow

and the complications that may arise.

Specific practical guides on management in a resource

poor country should include This includes half-hourly

applications of emollients, frequent oiling of skin, nurs-

ing in humidified incubator with careful temperature

monitoring, aseptic handling, investigation and treat-

Conflict of interest: None

Funding: None

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