Bullous ichthyosiform erythroderma

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 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 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. We report an 11-year-old boy with a generalized hyperkeratosis on the neck, trunk, extremities, and scalp.