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Progressive Systemic Scleroderma With IgA Deficiency in a Child

SUSAN JAY, MD; STEVEN HELM, MD; BETTY B. WRAY
Am J Dis Child. 1981;135(10):965-966. doi:10.1001/archpedi.1981.02130340069021.
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Connective tissue diseases that occur in childhood are frequently found in association with other disease processes. Recent studies have indicated that certain diseases, such as systemic lupus erythematosus, juvenile rheumatoid arthritis, and inflammatory myopathy, have been present in patients with an isolated IgA deficiency.1,2 We report an unusual case of progressive systemic scleroderma and IgA deficiency in a child.

Report of a Case.—This patient was a young girl whose early growth and development were appropriate. At the age of 3 years, she injured the skin overlying her right medial malleolus. A secondary infection subsequently developed at that site, and complete healing did not occur until five months later. A similar injury to her right knee at the age of 4 years took more than one year to heal and required hospitalization for wound care. By the age of 4 years 7 months, her weight and height had fallen

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