J Rheum Dis.  2019 Jul;26(3):211-218. 10.4078/jrd.2019.26.3.211.

Neuromyositis: A Rare Extramuscular Manifestation of Dermatomyositis

• Park CK
• Yoo SJ
• Yoo IS
• Kim J
• Shim SC
• Kang SW

• KMID: 2451514
• DOI: http://doi.org/10.4078/jrd.2019.26.3.211

Abstract

Dermatomyositis (DM) and polymyositis (PM) are representative idiopathic inflammatory myopathies characterized by symmetric and progressive proximal muscle weakness. Especially, DM is identified by characteristic skin lesions and has many extramuscular manifestations including various cardiac abnormalities, interstitial lung disease, and malignancy. However, involvement of peripheral nervous system in DM/PM is very rare and less known. The term "Neuromyositis" was introduced by Senator in 1893 to describe the concomitant involvement of the peripheral nervous system in DM/PM. Since then, a very few cases of neuromyositis have been reported mainly in the United States and Europe. Therefore, the pathogenetic mechanism and disease progression are unclear. In recent years, a few more cases were reported in Asia, specifically, China and Japan; however, none in Korea. Here, we describe a case of DM-associated neuromyositis in a 42-year-old man in Korea and review previous publications through literature research.

Keyword

Dermatomyositis; Peripheral nervous system diseases; Nerve conduction; Electromyography

MeSH Terms

Adult
Asia
China
Dermatomyositis*
Disease Progression
Electromyography
Europe
Humans
Japan
Korea
Lung Diseases, Interstitial
Muscle Weakness
Myositis
Neural Conduction
Peripheral Nervous System
Peripheral Nervous System Diseases
Polymyositis
Skin
United States

Figure

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  Figure 1. T2-weighted magnetic resonance images (A, B) and enhanced T1-weighted magnetic resonance image (C) showed high signal intensity in right su-praspinatus and anterior deltoid muscles (arrows). (D) Electron microscopy of right deltoid muscle biopsy demonstrated focal infiltration of fat globules, focal areas of myofibrillar loss, and several tubuloreticular structures (arrow) in the cytoplasm of endothelial cells, which were consistent with dermatomyositis (×30,000).

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  Figure 2. Initial chest computed tomography (CT) images showed multifocal patchy ground glass opacities (GGOs) in subpleural portion of both lower lungs (A, B). Follow-up chest CT images obtained 1 month after the initiation of therapy showed aggravation to reticulation with GGOs (C, D). Follow-up chest CT images obtained at the time of neuromyositis diagnosis showed improving state of previous noted reticulation and GGOs (E, F).

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