A Case of Anti-NXP2 Antibody-positive Juvenile Dermatomyositis with Characteristic Fascial Thickening on Muscle Ultrasound and Improvement with Immunotherapy

Koji Fukushima; Takeshi Yoshida; Hiroki Yamazaki; Naoko Takamatsu; Takashi Nagai; Yusuke Osaki; Masafumi Harada; Ichizo Nishino; Naoko Okiyama; Kazuma Sugie; Yuishin Izumi

doi:10.2169/internalmedicine.2720-23

This article has now been updated. Please use the final version.

A Case of Anti-NXP2 Antibody-positive Juvenile Dermatomyositis with Characteristic Fascial Thickening on Muscle Ultrasound and Improvement with Immunotherapy

Koji Fukushima, Takeshi Yoshida, Hiroki Yamazaki, Naoko Takamatsu, Takashi Nagai, Yusuke Osaki, Masafumi Harada, Ichizo Nishino, Naoko Okiyama, Kazuma Sugie, Yuishin Izumi

Author information

Koji Fukushima
Department of Neurology, Tokushima University Hospital, Japan
Department of Neurology, Nara Medical University School of Medicine, Japan
Takeshi Yoshida
Department of Rheumatology, Chikamori Hospital, Japan
Hiroki Yamazaki
Department of Neurology, Tokushima University Hospital, Japan
Naoko Takamatsu
Department of Neurology, Tokushima University Hospital, Japan
Takashi Nagai
Department of Pediatrics, Tokushima University Hospital, Japan
Yusuke Osaki
Department of Neurology, Tokushima University Hospital, Japan
Masafumi Harada
Department of Radiology, Tokushima University Hospital, Japan
Ichizo Nishino
Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Japan
Naoko Okiyama
Department of Dermatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Japan
Kazuma Sugie
Department of Neurology, Nara Medical University School of Medicine, Japan
Yuishin Izumi
Department of Neurology, Tokushima University Hospital, Japan

Keywords: Nuclear matrix protein 2, Juvenile dermatomyositis, Dermatomyositis sine dermatitis, Fasciitis, Myxovirus resistant protein A, Muscle ultrasound

JOURNAL OPEN ACCESS Advance online publication

Article ID: 2720-23

DOI https://doi.org/10.2169/internalmedicine.2720-23

The final version of this article is now available: Vol. 63 (2024), No. 12 pp. 1813-1817

Details

Abstract

We herein report a 12-year-old boy who presented with a fever, erythematous rash on the cheeks, back pain, and dysphagia. Blood tests revealed increased creatine kinase levels, and muscle ultrasonography (MUS) revealed characteristic fascial thickening in the lumbar paraspinal muscles, where myalgia was prominent. Sarcoplasmic expression of myxovirus-resistant protein A on a muscle biopsy and the presence of anti-nuclear matrix protein 2 (NXP2) antibodies confirmed the diagnosis of dermatomyositis. Prednisolone and intravenous immunoglobulin therapy improved the clinical and laboratory parameters as well as fascial thickening. MUS is useful for evaluating fasciitis associated with anti-NXP2 autoantibodies and monitoring therapeutic efficacy.

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