Juvenile systemic sclerosis with rapid progression and multisystem involvement in an adolescent.

Abstract

Juvenile systemic sclerosis (JSSc) is a rare, progressive autoimmune disorder that can affect the skin, musculoskeletal system, gastrointestinal tract, lungs, heart, kidney and endocrine glands. Clinical presentation varies depending on organs involved and may include oesophageal dysmotility, interstitial lung disease, pulmonary hypertension, constrictive pericarditis, patchy myocardial fibrosis and glomerulonephritis with considerable morbidity and mortality.We are reporting a rare case of JSSc with multisystem involvement in an adolescent male. He presented with a 5-month history of polyarticular arthritis, skin thickening and hypopigmented skin lesions. After 2 months of treatment with methotrexate, he reported minimal improvement in joint pain, swelling and skin thickening, followed by worsening after self-cessation of treatment due to varicella zoster infection. His condition progressed with worsening skin thickening, polyarticular arthritis, joint contractures, restricted mobility, interstitial lung disease and dysphagia.