Keratosis palmaris et plantaris cum surditate congenita et leuconychia totali unguium

Abstract

A case of keratosis palmoplantaris, transmitted as an irregular dominant, in a male age 13, deaf since birth, and with total leuconychia. Among 69 members of his family, 4 generations of which have been examined by the author, other signs of generalized keratosis, in the sense of the term used by Touraine, have been discovered. In the maternal collateral line were Dupuytren''s contracture and syndactyly; these disorders, while usually hereditary, could also be chance findings in this case. This is the first case described in the dermatological literature of the simultaneous appearance of keratotic lesions with congenital deafness and total leuconychia.