Weary et al. (1969) reported an apparently 'new' disorder characterized by generalized poikiloderma accentuated in flexural areas and on extensor surfaces, sclerosis of the palms and soles, and, in 1 patient, late development of subcutaneous calcification. Clubbing of ... Weary et al. (1969) reported an apparently 'new' disorder characterized by generalized poikiloderma accentuated in flexural areas and on extensor surfaces, sclerosis of the palms and soles, and, in 1 patient, late development of subcutaneous calcification. Clubbing of the fingers may be a feature. All 7 patients were black; 6 were from 1 family (mother and 5 affected children out of 10, by 3 different husbands) and a 4-year-old girl was affected in the other family. Weary (1982) reported that he knew of no more cases, but continued to be impressed with the uniqueness of the disorder on the basis of further observations on the 2 families. Fazio et al. (1995) reported the first case of hereditary sclerosing poikiloderma in a Caucasian. From Spain, Grau Salvat et al. (1999) described further cases in Caucasians and commented on new clinical features. Weary et al. (1969) had established 5 clinical features that defined the disorder. These are generalized poikiloderma with accentuation in flexural regions, sclerosis of palms and soles, linear hyperkeratosis and sclerotic bands in the axillae and antecubital and popliteal fossae, clubbing of the fingers, and tissue calcinosis as a late manifestation in 1 patient. Weary et al. (1969) reported noncalcified aortic stenosis of unknown etiology, without heart failure, in 1 of his patients. Grau Salvat et al. (1999) suggested that cardiac abnormalities may represent an important element of this disorder. In the family they reported, the proband was a 67-year-old woman in whom progressive generalized poikiloderma had first appeared at the age of 4 years. The mother and 2 brothers were also affected. The mother died at the age of 38 years of unknown causes, and the brothers died at the ages of 54 and 66 years as a result of valvular heart disease. Two sisters were healthy. Echocardiography in the proband showed severe aortic stenosis with heavily calcified valves, slight aortic regurgitation, and slight microstenosis and regurgitation with important thickening and calcification. All 3 affected sibs had prominent Raynaud phenomenon, vascular changes detected by digital capillaroscopy, but no finger clubbing.