case report collodion baby - a report of two cases

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Case Report COLLODION BABY - A REPORT OF TWO CASES. A. MOHANKAR*, S. KAR **, A. DONGRE *** ABSTRACT Two newborn presented in Neonatal ICU with parchment like membrane all over the body, ectropion, eclabian were also seen.History of consaugnity of marriage in parents was present. Collodion baby diagnoses was made. Its associated with Lamellar ichthyosis & non bullous ichthysioform erythroderma Key words :- Collodion baby, ectropion, eclabian, Lamellar ichthyosis & non bullous ichthysioform erythroderma Introduction Keratinization is a complex process in which the live, nucleated basal cells of the epidermis are transformed into dead, anucleated horny cells that form the outermost layer of epidermis - the stratum corneum 1 . Keratin, the fibrous protein forms the cytoskeletal component of the horny cells, sometimes when there is defect in gene, or gene mutation takes place then there may be disorders of keratinizatioin occurring 2 . Collodion baby - This is a descriptive term used for transient appearance of Neonates born with some form of congenital Ichthyosis 3 . The term Ichthyosis is derived from the Greek word 'ichthys' meaning fish and refers to the fish scale like appearance of skin. This is a disorder of keratinization or cornification resulting from abnormal epidermal differentiation or metabolism 4 . A newborn presented with tense skin all over the body with ectropion of the upper eyelids, eversion of upper lips (eclabian). The skin at birth, a parchment - thin yellowish, plastic like Case I Fig 1: Reported case on Day 1 Fig 2: Reported case on Day 14

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Page 1: Case Report COLLODION BABY - A REPORT OF TWO CASES

Case Report

COLLODION BABY - A REPORT OF TWO CASES. A. MOHANKAR*, S. KAR **, A. DONGRE ***

ABSTRACT

Two newborn presented in Neonatal ICU with parchment like

membrane all over the body, ectropion, eclabian were also seen.History of

consaugnity of marriage in parents was present. Collodion baby diagnoses

was made. Its associated with Lamellar ichthyosis & non bullous ichthysioform

erythroderma

Key words :- Collodion baby, ectropion, eclabian, Lamellar ichthyosis & non

bullous ichthysioform erythroderma

Introduction

Keratinization is a complex process in which the live, nucleated basal cells

of the epidermis are transformed into dead, anucleated horny cells that form the

outermost layer of epidermis - the stratum

corneum1.

Keratin, the fibrous protein forms the

cytoskeletal component of the horny cells,

sometimes when there is defect in gene, or gene

mutation takes place then there may be

disorders of keratinizatioin occurring2.

Collodion baby - This is a descriptive term

used for transient appearance of Neonates born

with some form of congenital Ichthyosis3.

The term Ichthyosis is derived from the

Greek word 'ichthys' meaning fish and refers to

the fish scale like appearance of skin. This is a

disorder of keratinization or cornification resulting

from abnormal epidermal differentiation or

metabolism4.

A newborn presented with tense skin all

over the body with ectropion of the upper eyelids,

eversion of upper lips (eclabian). The skin at

birth, a parchment - thin yellowish, plastic like

Case I

Fig 1: Reported case on Day 1

Fig 2: Reported case on Day 14

Page 2: Case Report COLLODION BABY - A REPORT OF TWO CASES

membrane covered the whole body, teethering at body orifices, limb

movements being restricted because of taught skin, Hairs & Nails were normal. History of

consanguinity of marriage of parents was present, on day 6, the culture from skin

showed growth of gram negative bacteria - Klebsiella species, the membrane

completely shed off on day 14th.

This newborn presented with parchment like membrane all over the body,

there is ectropion of upper eyelids, eclabian very prominent, associated with flat pinna,

sausage shaped digits, culture shows no growth. H/o consanguinity of marriage of

parents was present. Patient when reviewed on 6th day at a time of discharge

was seen to be shedding the membrane.

Discussion

In medical term collodioin baby applies to newborn who appear to have

an extra layer of skin (known as collodion membrane) that has a collodion like

quality. It is a descriptive term, used for transient appearance of neonate born

with some form of congenital ichthyosis.

The appearance is often described as shiny film looking like a layer of

Vaseline. The eyelids and mouth may have the appearance of being forced

open due to the tightness of the skin. There can be associated eversion of the

eyelids (ectropion), eversion of lip (eclabian), flat pinna, effacement of normal

skin creases and sausage shaped digits.

Most cases (apporxmately 75%) of collodian baby will go on to develop a

type of autosomal recessive congenital ichthyosis i.e. Lamellar ichthyosis & non

bullous congenital ichthysioform erythroderma.

In around 10% of cases the baby sheds this layer of skin and has normal

skin for the rest of its life. This is known as self-healing collodion baby.

The remaining 15% of cases are caused by a variety of diseases involving

keratinization disorders.

References

1. Dermatology - Moschella &Hurley - Volume 2 . 3rd Edition, Page no. 1385

2. Andrew's Diseases of Skin 9th Edition, Volume -1, Page No. 703.

3. Rooks Textbook of Dermatology - 7th Edition, Volume 1 , Page 14.20

4. IJDVL Year 1992, Volume : 58/Issue : 6/Page 393-394.