scleroderma in pediatric age group: report of 25 cases

scleroderma is a multisystem autoimmune connective tissue disease, cliaracterized by hardening of the skin. hv had 25 patients in the iranian pediatric unit of rheumatology over 10 years. there was female predominance if.m = 4/1, 80% female, 20% male). the age of onest was from 6 months to 16 years, the mean age being 10 years. the symptoms and signs were: nonpitting edema in 16%, liard skin in . sclerosis of distal limbs in 72%, facial in 20%, atrophy, ami hypopigmentation or hyperpigmentation in 40%, telangiectasis (15%), subcutaneous calcification (10%), raynaud's phenomenon in 60%, digital ulceration (16%), muscle pain and weakness, arthralgia (30%). rarer rnantfestantion included, dysphagia (16%), dyspnea (8%), cardiovascular disease, pericarditis, cardiomegafy, chf (16%). laboratory abnormalities included: anemia (80%), positive fan a. > 40 (88%) esr > 60 (40%) crp + + + (40%). skin biopsy documented increase in the collagen content of dermis in 75%.