Morbidity and Disability in Juvenile Localized Scleroderma: The Case for Early Recognition and Systemic Immunosuppressive Treatment

Juvenile localized scleroderma, also called morphea, is a chronic autoimmune and fibrosing disease that often causes severe damage in the growing child. Morbidities usually arise from extracutaneous involvement, and include joint contractures, hemiatrophy, seizures, and vision impairment. Studies have found extracutaneous involvement in 43%-70% of patients with juvenile localized scleroderma, 1 Wu E. Li S. Torok K. Virkud Y. Fuhlbrigge R. Rabinovich E. et al. Baseline description of the juvenile localized scleroderma subgroup from the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. ACR Open Rheumatol. 2019; 1: 119-124 Crossref PubMed Scopus (21) Google Scholar , 2 Kurzinski K.L. Zigler C.K. Torok K.S. Prediction of disease relapse in a cohort of paediatric patients with localized scleroderma. Br J Dermatol. 2019; 180: 1183-1189 Crossref PubMed Scopus (25) Google Scholar , 3 Li S.C. Li X. Pope E. Stewart K. Higgins G.C. Rabinovich C.E. et al. New Features for measuring disease activity in pediatric localized scleroderma. J Rheumatol. 2018; 45: 1680-1688 Crossref PubMed Scopus (18) Google Scholar , 4 Li S.C. Torok K.S. Rabinovich C.E. Dedeoglu F. Becker M.L. Ferguson P.J. et al. Initial Results from a pilot comparative effectiveness study of 3 methotrexate-based consensus treatment plans for juvenile localized scleroderma. J Rheumatol. 2020; 47: 1242-1252 Crossref PubMed Scopus (9) Google Scholar and functional impairment in over one-quarter of the 381 patients in a North American juvenile localized scleroderma registry. 1 Wu E. Li S. Torok K. Virkud Y. Fuhlbrigge R. Rabinovich E. et al. Baseline description of the juvenile localized scleroderma subgroup from the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. ACR Open Rheumatol. 2019; 1: 119-124 Crossref PubMed Scopus (21) Google Scholar