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• Vol.63 No.11
• CLINICA NEUROL, 63: 737−742, 2023
• Vol.63 No.11 contents

Case Report

Two cases of anti-nuclear matrix protein 2 antibody-positive dermatomyositis sine dermatitis with severe diffuse subcutaneous edema and dysphagia

Yoshiaki Takahashi, M.D.¹⁾, Motonori Takamiya, M.D.²⁾, Yuki Ichimura, M.D., Ph.D.³⁾, Naoko Okiyama, M.D., Ph.D.³⁾, Ichizo Nishino, M.D., Ph.D.⁴⁾ and Nobutoshi Morimoto, M.D.¹⁾

¹⁾ Department of Neurology, Kagawa Prefectural Central Hospital
²⁾ Department of Neurology, Okayama National Hospital Medical Center
³⁾ Department of Dermatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
⁴⁾ Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry

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.
Full Text of this Article in Japanese PDF (2621K)

(CLINICA NEUROL, 63: 737−742, 2023)
key words: dermatomyositis sine dermatitis, anti-nuclear matrix protein 2 (NXP-2) antibody, diffuse subcutaneous edema, dysphagia

(Received: 23-Mar-23)