Juvenile dermatomyositis, clinical manifestations and outcome in an Iranian cohort

Abstract

Objective: Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy of childhood and is characterized by proximal muscle weakness and pathognomonic skin rashes. In this study, we performed a descriptive cross sectional study to assess the clinical manifestations and outcomes of 39 patients with JDM from the northeast of Iran during 12 years and compared our findings with other studies. Design: 39 patients (16 boys and 23 girls) with juvenile dermatomyositis were studied retrospectively between 2001 and 2013. Gender, age at disease onset and diagnosis, clinical manifestations, laboratory data at onset, treatment and outcome of these patients were reviewed. Measurements and results: The mean age of onset was 9.42 ± 3.85 years. At the time of presentation, muscle weakness occurred in 100%; heliotrope rash in 51.2%; gottron’s papules in 46.1%, calcinosis in 12.8%, and 87.1% had at least one abnormal muscle enzyme result. Muscle biopsy was performed in 15.3% and was abnormal in all. All patients received corticosteroids; but methotrexate, hydroxychloroquine, intravenous immunoglobulin, or azathioprine was added to corticosteroid in some patients. The mean follow-up period was 22.66 ± 23.53 months. Conclusions: This study was in parallel with other reviews except for calcinosis which was observed with lower frequency. It is suggested that delay in diagnosis and treatment may be associated with calcinosis.

Keywords

• Juvenile dermatomyositis
• Inflammatory myopathy
• Skin manifestation
• Subcutaneous calcification