DERMATOMYOSITIS - A DERMATOLOGY-BASED CASE SERIES

Background: Dermatomyositis is associated with significant morbidity a nd occasional mortality. Currently there is no consensus on treatment for patients with dermatomyositis. Objective: Our purpose was to revie w the clinical features and response to therapy of patients with derma tomyositis and compare these data with previous series of patients wit h dermatomyositis/polymyositis. Methods: Clinical characteristics of 6 5 patients seen during a 10-year period were reviewed retrospectively. Twenty-one of these patients were enrolled in a prospective, uncontro lled study of treatment with high-dose prednisone followed by slow tap ering. Results: Clinical features were similar to those previously des cribed; however, muscle strength at diagnosis was on average greater i n patients in this series than in patients previously reported. Malign ancy was present in 5 of 43 adult patients (12%), but was not found in patients with juvenile dermatomyositis. Another connective tissue dis ease was present in 19% of patients. Twelve patients had dermatomyosit is sine myositis. Eighteen of 21 patients (85%) in the prednisone stud y group had resolution of myositis. Conclusion: Patients with dermatom yositis in this series had less active myositis at presentation, but w ere otherwise similar to patients with dermatomyositis/polymyositis pr eviously reported. Treatment with high-dose daily prednisone followed by slow tapering was effective.