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

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...

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Bibliographic Details
Published in:Internal Medicine 2024/06/15, Vol.63(12), pp.1813-1817
Main Authors: Fukushima, Koji, Yoshida, Takeshi, Yamazaki, Hiroki, Takamatsu, Naoko, Nagai, Takashi, Osaki, Yusuke, Harada, Masafumi, Nishino, Ichizo, Okiyama, Naoko, Sugie, Kazuma, Izumi, Yuishin
Format: Article
Language:English
Subjects:
Adenosine Triphosphatases
Autoantibodies
Autoantibodies - blood
Autoantibodies - immunology
Biopsy
Blood levels
Child
Creatine
Creatine kinase
Dermatomyositis
Dermatomyositis - diagnostic imaging
Dermatomyositis - drug therapy
Dermatomyositis - immunology
dermatomyositis sine dermatitis
DNA-Binding Proteins
Dysphagia
Fasciitis
Humans
Immunoglobulins, Intravenous - therapeutic use
Immunotherapy
Immunotherapy - methods
juvenile dermatomyositis
Male
Matrix protein
muscle ultrasound
Muscles
Myalgia
myxovirus resistant protein A
nuclear matrix protein 2
Prednisolone
Prednisolone - therapeutic use
RNA-Binding Proteins - immunology
Treatment Outcome
Ultrasonography
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Summary: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.
ISSN:0918-2918
1349-7235
1349-7235
DOI:10.2169/internalmedicine.2720-23