Normal sweating and tear production in congenital ichthyosiform erythroderma with deafness and keratitis

A 24-year-old female patient with congenital ichthyosiform erythroderma, deafness and vascularizing keratitis yet also examining normal sweating and tear production when tested is reported. This unique finding indicates that a certain subgroup of patients with this generalized ectodermal disturbance may benefit from treatment with the aromatic retinoids.