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.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003638:Deafness / Q000150:complications*; D005260:Female; D006801:Humans; D007057:Ichthyosis / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D007223:Infant; D007231:Infant, Newborn; D007637:Keratoconjunctivitis / Q000150:complications*; D012871:Skin Diseases / Q000150:complications; D013546:Sweating; D013577:Syndrome; D013666:Tears / Q000378:metabolism*