Sclerodrma in Iranian children and adolescents:Report of 35 cases

Abstract

Background: Scleroderma is a multisystem autoimmune connective tissue disease characterized by thickening and hardening of the skin.
Methods: Over 12 years we have observed 35 patients with scleroderma in the Pediatric Unit of Rheumatology. The diagnosis was established clinically and paraclinically in all patients.
Results: There was female predominance (F:M=2/5:1) (71% females, 29% males). The age of onset was 6 months to 16 years, the mean age being 10 years. Fever, fatigue, malaise were presented in 30-60%. The frequency of symptoms and signs was: nonpitting edema in 16%, hard skin in 60%, sclerosis of distal limbs in 72%, facial sclerosis in 15%, atrophy and hyper- or hypopigmentation in 40%, telangiectasis in 15%, subcutaneous calcification in 10%. Raynaud’s phenomenon in 60%, digital ulceration in 16%, muscle pain and weakness, and arthralgia in 30%. Rare manifestations included, dysphagia (16%), dyspnea (15%), cardiovascular disease, pericarditis, cardiomegaly, and CHF in 15%. Laboratory abnormalities included: anemia in 60%, positive F-ANA>1/160 (20%), ESR>50 (40%), CRP 3+ (40%). Skin biopsy documented increase in the collagen content of dermis in 75%.
Conclusion: Scleroderma is not rare disease in Iranian children in compared with European children, but it is more rare in comparison with adult.