Two patients with severe combined immunodeficiency (SCID) in whom cutaneous lesions were the first clinical feature were studied. Neither the morphology nor the histology of the lesions was uniform, although we have noted some common findings that can, in subsequent cases, lead us to suspect SCID. The immunologic defects were not uniform, representing the two poles of the spectrum of SCID. We believe that early recognition of the skin lesions is very important, since the patient's life expectancy can be increased by a bone marrow transplantation (1).