Juvenile dermatomyositis forty years on: Case report

Abstract

We present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications.

Juvenile Dermatomyositis Without Skin Lesions in an Antinuclear Matrix Protein 2 Antibody Seropositive Pediatric Case

“…5 Its reported incidence is 2–4 cases per million per year, mostly affecting women. 6 An alternative classification of the JIIM defines 2 classes of autoantibodies forming homogeneous groups of patients with similar clinical features, responses to therapy, and prognoses. These classes are myositis-associated autoantibodies present in patients with myositis and in patients with other autoimmune diseases, and MSAs found almost exclusively in patients with myositis.…”