Crest, or limited form of systemic sclerosis

A 12-year-old girl developed a white lump on the third finger of her right hand two years earlier. The lesion recurred after excision and a similar lump developed on the left knee and was also excised. Significant findings on physical examination included dilated capillaries in the nail beds and raised scaly violaceous lesions over the dorsal surface of the metacarpophalangeal joints. The skin on the fingers was hard (sclerodactyly) and there were some telangiectasias around the face and mouth and on the lips. Nail bed capillaries showed 'drop out' and dilatation, findings suggestive of dermatomyositis and scleroderma. The sclerodactyly pointed toward scleroderma. The absence of significant abnormalities in the lungs or the kidneys suggested the limited form of systemic sclerosis otherwise known by the old term CREST. There is no definitively effective treatment for scleroderma. Penicillamine may be effective. Nifedipine may be used for severe Raynaud's phenomenon. Calcinosis may respond to low dose warfarin therapy. Emollients for the skin are also used. Patients with this disease are at risk for GI problems, skin problems and Raynaud's phenomenon and pulmonary hypertension later in life.