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 atresia ani. The skin lesions were characterized by demarcated psoriasiform erythema with scaling. A skin biopsy revealed small abscesses containing polymorphonuclear leukocytes in the parakeratotic horny layer, elongation of the rete ridges and dermal papillae, and other findings consistent with psoriasis. A reverse-transcription polymerase chain reaction analysis disclosed increased expression of transforming growth factor alpha in the affected skin lesion of one of the siblings as well as in the skin of a patient with psoriasis. It is suggested that these cases are a variant of a congenital form of psoriasiform erythrokeratodermia.