Two first cousins with spondyloepiphyseal dysplasia tarda (X linked recessive form), one also with poikiloderma atrophicans vasculare progressing to lymphocytic lymphoma.

Two male cousins with spondyloepiphyseal dysplasia tarda (X linked recessive form) are described. One presented with a poorly differentiated lymphocytic lymphoma of the skin in areas of poikiloderma atrophicans vasculare which had been present for 10 years.