Juvenile Dermatomyositis is a rare idiopathic autoimmune and inflammatory myopathy and vasculopathy whose hallmarks are symmetrical proximal muscle weaknesses and a characteristic rash. Only few cases have been reported in West Africa subregion. We present a 14-year old Nigerian girl with clinical and histopathologic features of definitive juvenile dermatomyositis based on EULAR/ACR classification criteria but probable Juvenile dermatomyositis according to Bohan and Peter criteria.
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