Progressive partially symmetric erythrokeratodermia with deafness: histological and ultrastructural evidence for a subtype distinct from Schnyder's syndrome.

An atypical case of the rare genodermatosis erythrokeratodermia progressiva partim symmetrica (EPPS) with deafness and myopathy is described. Our findings suggest that this case of EPPS with deafness and myopathy represents a distinct entity of atypical erythrokeratodermias with characteristic epidermal and dermal skin changes.