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Definition

A skin disorder resembling bullous congenital ichthyosiform erythroderma. Affected individuals present with bullous ichthyosis in early childhood and hyperkeratotic lichenified plaques in the flexural areas and extensor surfaces at later ages. The feature that distinguishes AEI from BCIE is dramatic episodes of flares of annular polycyclic plaques with scale, which coalesce to involve most of the body surface and can persist for several weeks or even months.

Acronym

AEI

Synonyms

Annular ichthyosis variant of BCIE
Cyclic ichthyosis with epidermolytic hyperkeratosis

Keywords

Ichthyosis

 
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