A Case of Dermatomyositis Accompanied by Scleroderma

J Korean Neurol Assoc > Volume 8(1); 1990 > Article

Journal of the Korean Neurological Association 1990;8(1): 185-191.

공피증이 동반된 피부근염 1예

문준식, 김진호,이영진,이명식,최일생

연세대학교 신경과.

A Case of Dermatomyositis Accompanied by Scleroderma

Joon Shik Moon, M.D., Jin Ho Kim, M.D., Young Jin Lee, M.D., Myung Sik Lee, M.D., Il Saing Choi, M.D.,

Department of Neurology, Yongdong Severance Hospital, Yonsei University College of Medicine

Abstract

The idiopathic inflammatory myopathies form a heterogeneous group of disorders which differ in their severity, tempo of evolution, pattern of muscle involvement, associated clinicai features, pathological characteristics and response to therapy. According to the current literatures, dermatomyositis related with connective tissue disease constitutes 20% of all idiopathic inflammatory myopathies, but it seems that the case of dermatomyositis accompanied by scleroderma which is reported here is rare. We report a case of a 22-year oid female patient who was admitted to hospital due to progressive quadriparesis with deformity due to contracture and muscle atrophy of 1 year duration. There were whitish macules on the face and trunk : bilateral periorbital heliotrope rash : Gottron's papules on the dorsal aspect of the interphalangeal and metacarpo-phalangeal joints : sclerotic skin texture : and severe muscle atrophy and dysphagia. Laboratory examination revealed increaed creatine kinase, myopathic features were confirmed by electrophysiologic study, and subcutaneous calcification was seen on plain X-ray film. On skin and muscle biopsy, the characteristic features of dermatomyositis with scleroderma were seen.: dermal sclerosis, perifascicular atrophy, interstitial fibrosis and vasculitis. The patient died on the 28th hospital day due to sepsis.