AB1124 Juvenile localized scleroderma (JLS): Spectrum of clinical fenotypes and course

Abstract

Background jLS is an autoimmune fibrosing skin disorder. The skin lesions are polymorphic and may cause important aesthetic and functional sequels related to the involvement of skin and underlying tissues in a growing patient. Using the proposed classification 2004 Padua consensus conference we present a cohort of patients that reveals a wide spectrum of clinical manifestations and severity. Objectives Describe the clinical and laboratory characteristics of a group of patients with jLS in three medical centers in Bogotá and Cali, Colombia Methods Multicenter, retrospective study was conducted by collecting information of clinical and laboratory features, treatment and complications of patients with jLS Results This series includes 46 patients. Mean age at onset 7.4 years (2-15).Gender distribution Female 1, 5: Male 1. Mean time of follow-up was 37.2 months. 29% had family history of autoimmune disease. Mixed mophoea (presenting more than one type of lesions) was the most frequent subtype (41.3%), linear (17.4%), superficial circumscribed morphoea (19.6%), deep circumscribed morphoea(13%), generalized morphoea (4.3%),panclerotic morphoea (4.3%). 4/46 had arthritis at diagnosis. 5/46 developed other autoimmune disease: 1/5 had mixed morphoea, psoriasis and vitiligo, 2/5 localized scleroderma and Hashimoto’s disease, superficial circumscribed morphoea was a second autoimmune disease in a boy with juvenile dermatomiositis and in a girl with systemic lupus erythematosus. One patient with superficial morphoea on eyelid developed uveitis. 60% were antinuclear antibodies and 4, 2% were Anti-Scl70 positive. 95.6% were treated with methotrexate and all patients received topical therapy with steroids and/or tacrolimus. 15.2% received PUVA therapy. 8.7% were refractory to methotrexate and required another immunosuppressive medication. Complications: isolated aesthetic sequel (23.9%), limbs asymmetry associated joint functional restriction and aesthetic sequel (26.9%), facial deformity (19.6%), ulcerations, severe infection and limb amputation (2.2%). One patient required epiphysiodesis and soft tissues release. The mean referral time to the rheumatology clinic was 8. 6 months (1-60). Conclusions Localized scleroderma is a polymorphous disease, not benign that cause important morbidity. It requires early diagnosis and an aggressive treatment. A coordinate intervention of dermatologist and rheumatologist is desirable. References Laxer RM, Zulian F. Localized scleroderma. Current Opinion in Rheumatology 2006, 18:606–613 Zulian F, Vallongo C, Woo P, Russo R, Ruperto N, Harper J, et al. Localized Scleroderma in Childhood Is Not Just a Skin Disease. Arthritis and Rheumatism 2005;52:2873-2881 Zulian F, Martini G, Vallongo C, Vittadello F, Falcini F, Patrizi A, et al. Methotrexate Treatment in Juvenile Localized Scleroderma. A Ramdomized, Double-Blind, Placebo-Controlled Trial.Arthritis and Rheumatism 2011; 63: 1998-2006 Disclosure of Interest None Declared