CONGENITAL PSORIASIFORM ERYTHROKERATODERMIA WITH CLEIDOCRANIAL DYSPLASIA, UROGENITAL ANOMALIES AND ATRESIA ANI

We describe two siblings with unique psoriasiform erythrokeratodermia associated with cleidocranial dysplasia, urogenital anomalies and atre sia ani. The skin lesions were characterized by demarcated psoriasifor m erythema with scaling. A skin biopsy revealed small abscesses contai ning polymorphonuclear leukocytes in the parakeratotic horny layer, el ongation of the rete ridges and dermal papillae, and other findings co nsistent with psoriasis. A reverse-transcription polymerase chain reac tion analysis disclosed increased expression of transforming growth fa ctor a in the affected skin lesion of one of the siblings as well as i n the skin of a patient with psoriasis. It is suggested that these cas es are a variant of a congenital form of psoriasiform erythrokeratoder mia.