[MIM*124500] diffuse keratoderma of the extremities, with the development during childhood of constricting fibrous bands around the middle phalanx of the fingers or toes that may lead to spontaneous amputation; there may be congenital deafness; autosomal dominant inheritance, caused by mutation in the gene for loricrin (LOR), a component of the epidermal differentiation complex on 1q. SYN: keratoma hereditarium mutilans, Vohwinkel syndrome.