Juvenile Dermatomyositis: Case Report from the Albert Royer National Children's Centre in Dakar (Senegal)

Author(s): Yaay Joor Koddu Biigé DIENG*, Amadou SOW, Djénaba Fafa CISSE, Fatime TALL, Idrissa Demba BA, Papa Moctar FAYE, Ousmane NDIAYE

Juvenile dermatomyositis (JD) is a rare multi-systemic inflammatory idiopathic disease characterised by primary cutaneous and muscular involvement. There is a paucity of literature concerning children. We report the case of a 7-year-old girl. The diagnosis was made following hospitalisation for respiratory distress with a fever of 39°C and deterioration in general condition with general muscle wasting and relative functional impotence. Examination revealed flaccid quadriplegia, significant muscular amyotrophy and ankylosis with pain on mobilisation of the joints. Hypochromic macules associated with Gottron papules were also found. Biochemistry revealed elevated muscle enzymes and LDH. EMG revealed myogenic patterns and muscle biopsy revealed inflammatory infiltrates in the endomysium and connective tissue. Treatment included prednisone, methotrexate and folic acid. The short- and medium-term outcome was favourable. Death occurred 20 months later at home from an unclear cause.

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