KID syndrome (keratitis, ichthyosis, and deafness)

Keratitis, ichthyosis and deafness are the dominant signs of KID syndrome. The lesions involving cornea epidermis and internal ear are probably the re sult of a congenital ectodermal abnormality. Associated signs such as incre ased sensitivity to infections, and dermoskeleton dystrophies are also usef ul for the diagnosis. There are no specific biological signs. Most cases ar e sporadic but familial cases have been described with unclear mode of inhe ritance. Treatment is disappointing. Thus management mainly relies upon ear ly detection of complications. (C) 1999 Elsevier, Paris.