[Clinical characteristics of juvenile dermatomyositis].

INTRODUCTION

Dermatomyositis, belonging to the group of the idiopathic inflammatory myopathies, is characterized by bimodal pattern of age-specific incidence of rates, with peaks in age group from 5 to 14 years (juvenile dermatomyositis) and in age group from 45 to 64 years (adult dermatomyositis).

THE AIM OF THIS STUDY: Is to evaluate the clinical characteristics of 12 patients with juvenile dermatomyositis followed by the 3rd Department of Internal Medicine, University of Debrecen and the 2nd Department of Pediatrics, Semmelweis University, Budapest.

METHODS

The authors analyzed the medical records of the patients with juvenile and adult form of dermatomyositis retrospectively.

RESULTS

All of the children had symmetrical weakness of the proximal muscles. The most frequent cutaneous features were facial erythema and Gottron papules (11/12). The extramuscular manifestations were also assessed. 7 children had arthralgia. There were observed pulmonary fibrosis, Raynaud-syndrome, dysphagia and sicca-syndrome in the same patient, whose disease is overlapped with progressive systemic sclerosis. In view of the clinical course, the authors found that prevalence of polycyclic (relapsing-remitting) and monophasic subtypes of the disease were similar (6/12 and 5/12). Finally, all of the patients achieved remission, however, 2 patients have to take low-dose corticosteroid therapy permanently to maintain remission. One patient's cutaneous symptoms proved to be persistent and in further 2 cases, regression of the calcinosis is slow, but continuous.

DISCUSSION: The authors compare their data of juvenile patients with the data of the relevant literature and to their experience with the management of adult DM patients. It seems to be reasonable to treat the patients in centres.