Bullous ichthyosiform erythroderma: case presentation

ABSTRACT

Bullous Ichthyosiform Erythroderma [BIE] is a rare disorder of keratinization [mutation 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 scale builds up in skin creases, sometimes forming spiny [Hystrix] outgrowths. Cobblestone-like keratoses occur at other sites such as the dorsal 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