Bullous ichthyosiform erythroderma

Abstract

Bullous ichthyosiform erythroderma (BIE) is a rare disorder of keratinization (mutations in either keratin 1 or 10). It typically presents with fragile skin, which gives way to gradual evolution<br />of hyperkeratosis. Flaccid blisters, peeling, and superficial erosions at sites of minor trauma or friction are apparent within the first few hours of life. Yellow-brown, waxy, ridged or corrugated<br />scales build up in skin creases, sometimes forming spiny (Hystrix) outgrowths. Cobble stone-like keratoses occur at other sites such as the dorsal aspects of the hands and feet and over the trunk.<br />We report an 11-year-old boy with a generalized hyperkeratosis on the neck, trunk, extremities, and scalp.