Atypical ichthyosiform erythroderma, deafness and keratitis.

Abstract

Two patients with ichthyosiform erythroderma of the same unusual but characteristic distribution are described. Both patients were born with perceptive deafness and developed severe vascularizing keratitis in early childhood. There is no family history of the disorder in either case. This syndrome is discussed in relation to previous reports of atypical ichthyosiform erythroderma associated with deafness.