title: Localized severe scleroderma: a retrospective study of 26 pediatric patients.
abstract: Juvenile localized scleroderma includes different conditions characterized by skin hardening with increased collagen deposition. Although juvenile localized scleroderma is considered a relatively benign disease, lesions may extend through the dermis, subcutaneous tissue, muscles, and the underlying bone, leading to significant functional and cosmetic deformities. Furthermore, extracutaneous manifestations are described. We retrospectively analyzed a cohort of 26 patients with severe Juvenile localized scleroderma with particular attention to clinical features, therapy, and long-term outcome. A subgroup of three patients has been further evaluated with infrared thermography. Our findings were consistent with the current literature for demographic, laboratory, and clinical characteristics at disease onset, but, with our patients, the prevalence of extracutaneous manifestations was higher, thus confirming the potential for severe juvenile localized scleroderma to affect organs other than the skin, without increased risk of development toward systemic sclerosis. Correlation between various treatments and clinical endpoint showed that systemic therapy lead to a better outcome: in particular, methotrexate appeared the most effective drug, capable in halting the progression of the disease and sometimes inducing its regression.
authors.affiliation: II Pediatric, Fondazione Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, IRCCS, Milan, Italy. mabel75@libero.it
authors.forename: Matilde
authors.initials: M
authors.lastname: Beltramelli
authors.forename: Paolo
authors.initials: P
authors.lastname: Vercellesi
authors.forename: Adina
authors.initials: A
authors.lastname: Frasin
authors.forename: Carlo
authors.initials: C
authors.lastname: Gelmetti
authors.forename: Fabrizia
authors.initials: F
authors.lastname: Corona
mesh_terms: D000293:Adolescent; D017153:Antibodies, Anticardiolipin / Q000097:blood; D018501:Antirheumatic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004643:Emollients / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D010396:Penicillamine / Q000627:therapeutic use; D012189:Retrospective Studies; D012594:Scleroderma, Localized / Q000276:immunology / Q000473:pathology* / Q000628:therapy*; D012720:Severity of Illness Index; D013256:Steroids / Q000627:therapeutic use*; D013817:Thermography; D016896:Treatment Outcome
chemical_list: D017153:Antibodies, Anticardiolipin; D018501:Antirheumatic Agents; D004643:Emollients; D013256:Steroids; D010396:Penicillamine; D008727:Methotrexate