Hereditary sclerosing poikiloderma. Report of two families with an unusual and distinctive genodermatosis

A new heritable disorder of connective tissue is described in seven members of two unrelated Negro families. The clinical features of this disorder which are clearly established are: A remarkable generalized poikiloderma with marked accentuation in the skin of the flexural regions of the axilla, antecubital, and popliteal fossae and of the extensor surfaces of the knees, elbows, and joints of the hands. Sclerosis of the palms and soles unlike that seen in scleroderma. Linear and reticulated hyperkeratotic and sclerotic bands in the axilla and the antecubital and popliteal fossae. Clubbing of the fingers. Calcinosis of the tissues as a late manifestation in one patient. Histologic examination of tissue from the antecubital fossa and axilla reveals focal homogenization of collagen and diminished elastic tissue.