Juvenile Dermatomyositis: Diagnosis and Management.

Abstract

Juvenile dermatomyositis (JDM) is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Seven patients with JDM found in the records from 1998-2019 of the Department of Dermatology Farhat Hached Hospital, Sousse, Tunisia. Our study concerned a total of six girls and one boy with a median age at disease onset of 8,16 years. The average time before diagnosis was 8,8 months. The onset of the disease was acute in 2 patients. All patients displayed skin manifestations at diagnosis, with proximal muscular weakness in 4 cases. Four patients had elevated muscle enzymes and all of them showed myopathic findings on electromyography. Oral corticosteroids were prescribed in 6 patients, in association with other systemic therapies. Three patients achieved a good outcome while two others relapsed. The two other patients showed corticosteroids resistance with a fatal outcome in one case. This study highlights the diagnostic features and management of juvenile dermatomyositis.