Chronic Inflammatory Demyelinating Polyneuropathy Combined With
Limited Cutaneous Systemic Sclerosis

J Korean Neurol Assoc > Volume 32(1); 2014 > Article

Journal of the Korean Neurological Association 2014;32(1): 26-29.

제한전신경화증 환자에서 발생한 만성염증탈수초다발신경병

이은경, 윤동주 고영채 장상현 윤수진 오건세 이수주 박성혜

을지대학교 의과대학 신경과학교실, 서울대학교 의과대학 병리학교실

Chronic Inflammatory Demyelinating Polyneuropathy Combined With Limited Cutaneous Systemic Sclerosis

Eun Kyung Lee

Department of Neurology, Eulji University School of Medicine, Daejeon, Korea Department of Pathology a , Seoul National University College of Medicine, Seoul, Korea

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) has been rarely reported in systemic sclerosis (SSc). We describe the clinical, electrophysiological, and pathologic findings in a 73-year-old female with established limited cutaneous SSc who later developed CIDP. The patient had progressive limb weakness, sensory loss, and slow nerve conduction velocities. Sural nerve biopsy showed evidence of demyelination and remyelination. This case demonstrates that chronic demyelinating polyneuropathy can be an unusual manifestation of SSc, presumably resulting from an immune-mediated process. Key Words: Chronic inflammatory demyelinating polyneuropathy, Systemic sclerosis, Autoimmunity