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Cuoco F., Petaccia A., Corona F.
UOS Reumatologia Pediatrica, Clinica Pediatrica De Marchi, IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano, Italia
Juvenile dermatomyositis is an autoimmune idiopathic inflammatory myopathy associated with systemic vasculopaty. Its main characteristics are specific vasculitic changes in the skin and inflammation of the muscle, causing progressive muscle weakness. The presenting features may include a disease pattern of an erythematous rash on the cheeks, a red to violacious rash on the eyelids with edema, raised red papules on the extensor surfaces of finger joints, abnormal nailbed capillaries and a red rash on the elbows, knees and ankles. The hallmark feature is proximal muscle weakness that may be severe and debilitating. We present a case of a 4-year-old boy with skin rash and muscle weakness. Abnormal laboratory findings with elevation of muscle enzymes, pathologic electromyogram and clinical manifestations led to establish diagnosis of juvenile dermatomyositis. The child started treatment with methylprednisolone pulse therapy and oral prednisone with improvement. During the hospitalization he presented exacerbation of the disease, which needed the modification of steroid therapy in place. It was not necessary to use other treatment, like methotrexate or cyclosporine. Is therefore stresses the validity of steroid therapy as first line treatment in juvenile dermatomyositis, without necessarily having to resort to additional drugs.