Document Detail

Localized scleroderma in childhood is not just a skin disease.
MedLine Citation:
PMID:  16142730     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma. METHODS: Data from a multinational study on juvenile scleroderma was used for this in-depth study. Clinical features of patients with extracutaneous manifestations were compared with those of patients who had exclusively skin involvement. RESULTS: Seven hundred fifty patients entered the study. One hundred sixty-eight patients (22.4%) presented with a total of 193 extracutaneous manifestations, as follows: articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%), and renal (1%). Other autoimmune conditions were present in 7.3% of patients. Neurologic involvement consisted of epilepsy, central nervous system vasculitis, peripheral neuropathy, vascular malformations, headache, and neuroimaging abnormalities. Ocular manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papilledema. In more than one-fourth of these children, articular, neurologic, and ocular involvements were unrelated to the site of skin lesions. Raynaud's phenomenon was reported in 16 patients. Respiratory involvement consisted essentially of restrictive lung disease. Gastrointestinal involvement was reported in 12 patients and consisted exclusively of gastroesophageal reflux. Thirty patients (4%) had multiple extracutaneous features, but systemic sclerosis (SSc) developed in only 1 patient. In patients with extracutaneous involvement, the prevalence of antinuclear antibodies and rheumatoid factor was significantly higher than that among patients with only skin involvement. However, Scl-70 and anticentromere, markers of SSc, were not significantly increased. CONCLUSION: Extracutaneous manifestations of juvenile localized scleroderma developed in almost one-fourth of the children in this study. These extracutaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc was very low. This subgroup of patients with juvenile localized scleroderma should be evaluated extensively, treated more aggressively, and monitored carefully.
Francesco Zulian; Cristina Vallongo; Patricia Woo; Ricardo Russo; Nicolino Ruperto; John Harper; Graciela Espada; Fabrizia Corona; Masha Mukamel; Richard Vesely; Elzbieta Musiej-Nowakowska; Jeff Chaitow; Joan Ros; Maria T Apaz; Valeria Gerloni; Henryka Mazur-Zielinska; Susan Nielsen; Susanne Ullman; Gerd Horneff; Carine Wouters; Giorgia Martini; Rolando Cimaz; Ronald Laxer; Balu H Athreya;
Publication Detail:
Type:  Journal Article; Multicenter Study    
Journal Detail:
Title:  Arthritis and rheumatism     Volume:  52     ISSN:  0004-3591     ISO Abbreviation:  Arthritis Rheum.     Publication Date:  2005 Sep 
Date Detail:
Created Date:  2005-09-12     Completed Date:  2005-11-01     Revised Date:  2006-11-08    
Medline Journal Info:
Nlm Unique ID:  0370605     Medline TA:  Arthritis Rheum     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2873-81     Citation Subset:  AIM; IM    
Dipartimento di Pediatria, Università di Padova, Padua, Italy.
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MeSH Terms
Autoimmune Diseases / complications,  diagnosis,  epidemiology
Cohort Studies
Eye Diseases / complications,  diagnosis,  epidemiology
Gastrointestinal Diseases / complications,  diagnosis,  epidemiology
Heart Diseases / complications,  diagnosis,  epidemiology
Joint Diseases / complications,  diagnosis,  epidemiology
Kidney Diseases / complications,  diagnosis,  epidemiology
Nervous System Diseases / complications,  diagnosis,  epidemiology
Respiratory Tract Diseases / complications,  diagnosis,  epidemiology
Retrospective Studies
Scleroderma, Localized / complications*,  diagnosis,  epidemiology
Vascular Diseases / complications,  diagnosis,  epidemiology
Comment In:
Arthritis Rheum. 2006 Sep;54(9):3065-6; author reply 3066   [PMID:  16948151 ]

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