AB1124 Juvenile localized scleroderma (JLS): Spectrum of clinical fenotypes and course
Authors: C. Malagon, C. Mosquera, P. Gomez
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> 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. <h3>Objectives</h3> Describe the clinical and laboratory characteristics of a group of patients with jLS in three medical centers in Bogotá and Cali, Colombia <h3>Methods</h3> Multicenter, retrospective study was conducted by collecting information of clinical and laboratory features, treatment and complications of patients with jLS <h3>Results</h3> 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). <h3>Conclusions</h3> 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. <h3>References</h3> 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 <h3>Disclosure of Interest</h3> None Declared