Bullous Congenital Ichthyosiform Erythroderma in Japan: A Nationwide Survey

Findings include discovery of a notable sex difference in the clinical manifestation of BCIE scales.

Bullous congenital ichthyosiform erythroderma (BCIE), also known as “epidermolytic hyperkeratosis,” is a rare autosomal dominant genodermatosis. BCIE may present at birth as collodion baby, or with erythema, scales, blisters, or erosions. In childhood, blistering decreases but thick ichthyotic or hystrix scales develop. Palmar plantar keratoderma (PPK) and generalized malodor are common.

These authors surveyed 802 dermatology departments in Japan and identified an estimated 55 patients with BCIE who were seen in 2002 (35 male). Clinical information was available in 28 cases. No geographic or sex predilection was evident. A family history of BCIE was identified in 3 cases (11%). “Rash” (96%), erythroderma (68%), and blistering (generalized in…

Reviewing Author

Mary Wu Chang, MD

Disclosures

Consultant / Advisory board Pierre Fabre; Valeant

Speaker’s bureauPierre Fabre

Mary Wu Chang, MD

Disclosures

Consultant / Advisory board Pierre Fabre; Valeant

Speaker’s bureauPierre Fabre