E38 Pediatric dermatomyositis, what specificities

Abstract

Abstract Introduction Dermatomyositis is a rare disease. In children the incidence is 2–3 per million population. It is a potentially serious disease whose mortality is < 5%, but whose complications and long-term prognosis remain poor for some children. Objectives We report the epidemiological, clinical, therapeutic and evolutionary characteristics of a retrospective case series of 14 children with dermatomyositis seen at the pediatric center of Setif (Algeria). Materials and methods The average age of onset is 8 years (0–15 ½ years), The sex ratio (M/F) was 0.21; The mean duration of symptoms prior to diagnosis was 4.4 months. At the time of diagnosis, 100% of children had skin rash, 84% eyelid telangiectasia, 40% Gottron's papules, 90% muscle weakness, 40% arthritis, 13% fever, and 25% dysphagia. The muscle enzymes were elevated in all, FAN positive in 6 cases, anti-DNA was positive in a single patient with overlapping myositis. The electromyogram cames myogenic for all our 14 patients, 10 patients had muscle biopsy with report of non-specific interstitial and parenchymal myositis Therapeutic regimens were variable, they depended on the severity of the initial picture, Nevertheless all of the children were treated with first-line corticosteroids (100%), methotrexate was prescribed in 12 cases (85%); Mycophenolate mofetil and Rituximab in one patient, and bisphosphonate in a single child with extensive calcinosis. Polyvalent immunoglobulins were used for 4 cases with swallowing disorders and calcinosis (28%), 4 patients had calcinosis as complication (28%), only one child died from aspiration pneumonitis. Discussion The diagnosis is made according to the criteria of Bohan and Peter, given the non-specificity of the muscle biopsies. The clinical and evolutionary characteristics of our serie are consistent with the overall data in the literature with a predominance of skin and muscle involvement. However, some specificities were individualized such as the case of myositis overlapping with lupus. The treatment is essentially based on corticosteroid therapy and methotrexate. Conclusion Juvenile dermatomyositis is a rare serious and chronic condition requiring multidisciplinary management. Ethics Associate Professor of pediatrics, department of medicine University Setif-Algeria (pediatric center –Setif-Algeria)