학술논문

Two cases of anti-nuclear matrix protein 2 antibody-positive dermatomyositis sine dermatitis with severe diffuse subcutaneous edema and dysphagia / 著明なびまん性皮下浮腫と嚥下障害を呈した抗nuclear matrix protein 2抗体陽性皮膚炎欠如型皮膚筋炎の2例
Document Type
Journal Article
Source
臨床神経学 / Rinsho Shinkeigaku. 2023, 63(11):737
Subject
anti-nuclear matrix protein 2 (NXP-2) antibody
dermatomyositis sine dermatitis
diffuse subcutaneous edema
dysphagia
びまん性皮下浮腫
嚥下障害
抗NXP-2抗体
皮膚炎欠如型皮膚筋炎
Language
Japanese
ISSN
0009-918X
1882-0654
Abstract
Case 1 involved a 68-year-old woman who was admitted to our hospital because of muscle weakness, diffuse subcutaneous edema, dysphagia, and an elevated serum creatine kinase level that had worsened within the previous month. Case 2 involved a 78-year-old woman who was admitted to our hospital because of muscle weakness, bilateral shoulder pain, diffuse subcutaneous edema, and dysphagia that had gradually worsened during the past 5 months. Both patients showed severe diffuse subcutaneous edema and dysphagia and underwent enteral tube feeding. Although they had no skin lesions consistent with dermatomyositis, muscle biopsies showed myxovirus resistance protein A (MxA) expansion, and blood tests showed positivity for anti-nuclear matrix protein 2 (anti-NXP-2) antibody. Therefore, both presents were diagnosed with anti-NXP-2 antibody-positive dermatomyositis sine dermatitis (DMSD). Anti-NXP-2 antibody-positive dermatomyositis has been reported to be closely associated with DMSD, severe edema and dysphagia. Differential diagnosis for patients who develop myositis with severe subcutaneous edema and dysphagia should include anti-NXP-2 antibody-positive dermatomyositis, and it is important to consider measurement of anti-NXP-2 antibody.