SCLERODERMA IN PEDIATRIC AGE GROUP: REPORT OF 25 CASES

Abstract

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%.