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Image Number 531 (Congenital ichthyosiform erythroderma)


Diagnosis: Congenital ichthyosiform erythroderma

Site: Axillae

Sex: F

Age: 4

Type: Clinical

Description: Erythematous scaly plaques

Submitted by: Shahbaz Janjua

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Differential Diagnosis

History: Since the 1980s, nonbullous autosomal recessive ichthyoses have been divided into two major clinical entities, nonbullous congenital ichthyosiform erythroderma (NBCIE) and lamellar ichthyosis.  As many as 90% the individuals affected with NBCIE present at birth as collodion babies but ectropion, eclabion, scalp involvement, and loss of eyebrows and lashes seem to be more frequent in non bullous congenital ichthyosiform erythroderma than in lamellar ichthyosis. 

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