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Sarcoidosis is rare in young children, and is characterized by skin, joint, and eye changes. Differentiated clinically from juvenile rheumatoid arthritis (JRA) by milder constitutional symptoms and characteristic joint abnormalities, sarcoidosis is confirmed by demonstrating noncaseating granulomas in skin, conjunctival, or synovial biopsies. Recent reports have shown children with features of both sarcoidosis and juvenile rheumatoid arthritis, some with similarly affected family members. We cared for four children with sarcoidosis and severe joint manifestations. Two had a personal or family history of JRA. Three of the four children had ichthyosiform cutaneous manifestations, which may suggest an association between severe joint disease and ichthyosiform changes. Because of the difficulty in making a diagnosis on clinical grounds alone, biopsy of cutaneous lesions is recommended in children with these symptoms.

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