Juvenile dermatomyositis: a 8 cases report

ABSTRACT

Juvenile dermatomyositis is a multisystemic rheumatic disorder characterized by non infectious inflammation of skin and striated muscle. The aim of this retrospective study is to stress the diagnostic and therapeutic difficulties of dermatomyositis in children. From January 1990 to December 2002, 8 cases of childhood dermatomyositis were collected in the Dermatology Department of Casablanca University Hospital. The mean age of our patient was 10 year-old [3 to 14 year-old]. They were 6 girls and 2 boys. The evolution mean duration was 17 months. Cutaneous and muscular signs were found in all cases. Raynaud's phenomenon was present in 2 cases. Two children had bad general status and 1 child had a dysphagia. An inflammatory syndrome was present in all cases. Muscle enzymes were increased in 6 patients [75%]. Muscular biopsy has confirmed the diagnosis in 5 cases [62%]. High dose corticosteroid therapy was used in all cases, associated to immunosuppressive therapy in 1 case. The evolution was good in 2 cases. Three children had a relapse with cutaneous calcinosis in 2 cases. Three patients were lost from follow-up. Juvenile dermatomyositis is a severe disease. Corticosteroids, associated to immunosuppressors or immunoglobulins have decreased mortality. The prognosis depends on an early diagnosis and treatment