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Submitted: 18 January 2012 Modified: 10 May 2013
HERDIN Record #: NCR-JRRMMC-01181202015556

A case report of Lamellar ichtyosis in a Filipino infant


CONTEXT:
Ichthyosis is the name given to a group of disorders characterized by a generalized persistent non-inflammatory scaling disorder of the skin surface. The term derives from a fanciful likeness of the skin surface to fish skin, although the scale of most fish is mesodermal rather than ectodermal in origin.

A collodion baby is an infant born with a tough, parchment-like membrane covering that subsequently desquamates over several days leaving large, platelike scales and depending on the persistence of erythema develops into one of the ichthyosiform disorders

OBJECTIVE:
The purpose of this paper is to describe a rare case of a collodion baby and to document her full expression to lamellar ichthyosis (LI).

STUDY DESIGN: Case Report

STUDY SETTING: Tertiary Government Hospital (JRRMMC)

STUDY POPULATION: Two-day old infant, with erythroderma, dry, cracked skin, ectropiono and eclabium.

RESULTS:
A two-day old infant born with a collodion membrane with erythoderma, generalized scaling, ectropion, eclabion and eclabium was described. Histophatologic findings were consistent with lamellar ichtyosis. Her evolution to lamellar ichthyosis was documented.

CONCLUSION:
Although rare, every primary health care worker should be able to recognize a collodion baby and institute immediate care to prevent dehydration, infection, keratitis and problems with feeding. Understanding the role genetics play in lamellar ichthyosis particularly its 25% chance to be inherited in each pregnancy is integral to parent education.

Publication Type
Research Report
Date

Objectives

General Objective:
To describe a rare case of lamellar ichthyosis in a Filipino infant.

LocationLocation CodeAvailable FormatAvailability
Medical Library, 5/F Training and Research Office, Jose R. Reyes Memorial Medical Center 00-1 #2 Fulltext Print Format

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