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• Vol.64 No.6
• CLINICA NEUROL, 64: 417−421, 2024
• Vol.64 No.6 contents

Case Report

Juvenile-onset anti-nuclear matrix protein 2 (NXP-2) antibody-positive dermatomyositis with joint contractures before manifestation of myositis: a case report

Eito Miura, M.D.^1）, Tomone Taneda, M.D.^1）, Yoshitaka Umeda, M.D.^1）, Maiko Umeda, M.D.^1）, Mutsuo Oyake, M.D., Ph.D.^1）, Takashi Matsushita, M.D., Ph.D.^2）, Ichizo Nishino, M.D., Ph.D.^3） and Nobuya Fujita, M.D., Ph.D.^1）

^1）Department of Neurology, Nagaoka Red Cross Hospital
^2）Department of Dermatology, Kanazawa University
^3）Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP)

A 23-year-old man was admitted to our hospital with a one-year history of muscle weakness and atrophy. He had noticed contractures of the fingers of both hands from the age of 18. Examination revealed a skin rash including heliotrope rash and Gottron's sign, joint contractures in the extremities, dysphagia, extensive muscle weakness and marked muscle atrophy. The serum creatine kinase level was 272 IU/l and muscle biopsy showed typical perifascicular atrophy but little lymphocyte invasion. There was no interstitial pneumonia or malignancy, but muscle tendons showed elevated CT values suggesting calcification or fibrosis. Anti-nuclear matrix protein 2 (NXP-2) antibody-positive dermatomyositis was diagnosed on the basis of the serum antibody level. Methylprednisolone pulse therapy ameliorated the skin rash and bulbar palsy, but muscle weakness, atrophy and joint contractures were resistant to the treatment. There have been no previous reports of young adults with anti-NXP-2 antibody-positive dermatomyositis in whom joint contracture became evident as early as 4 years beforehand, which is a important feature for differential diagnosis of dermatomyositis.
Full Text of this Article in Japanese PDF (2366K)

(CLINICA NEUROL, 64: 417−421, 2024)
key words: anti-nuclear matrix protein 2 (NXP-2) antibody, dermatomyositis, joint contracture, juvenile onset

(Received: 11-Jan-24)