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A case of eosinophilic fasciitis without skin manifestations: a case report in a patient with lupus and literature review

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Abstract

Eosinophilic fasciitis (EF) is a rare connective tissue disease that causes inflammation and fibrosis of the fascia, inducing pain and motor dysfunction. Characteristic skin manifestations, such as edema, erythema, induration, peau d’orange appearance, and the groove sign, are of diagnostic significance and observed in the majority of patients with EF. We herein report a case of EF without these characteristic skin manifestations. A 66-year-old Japanese woman developed progressive limb pain and motor dysfunction. No skin changes were observed. We diagnosed the patient with EF based on the clinical course, magnetic resonance imaging, and en bloc biopsy containing fascia and muscle. Oral prednisolone therapy markedly attenuated limb pain and motor dysfunctions. Through a systemic search of the medical literature, we retrieved 4 juvenile cases and 8 adult cases of EF without characteristic skin manifestations during the clinical course. We herein present a systemic review on EF without skin manifestations and discuss differences between the two proposed sets of diagnostic criteria of EF.

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Data availability

The datasets obtained and/or analyzed during the current study available from the corresponding author on reasonable request.

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Acknowledgments

We would like to thank Professor Masataka Kuwana (Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan) for performing serum anti-ARS antibody tests, Dr. Sho Nishikawa and Professor Masayuki Iwano (Department of Nephrology, Faculty of Medical Sciences, University of Fukui, Fukui, Japan) for evaluating renal biopsy specimens, and Dr. Hiroshi Kasamatsu (Department of Dermatology, Faculty of Medical Sciences, University of Fukui, Fukui, Japan) for obtaining en bloc biopsy specimens.

Funding

This study was supported partly by Intramural Research Grant (2-5 and 29-4) for Neurological and Psychiatric Disorders of NCNP.

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Authors and Affiliations

  1. Faculty of Medical Sciences, University of Fukui, Fukui, Japan

    Kohei Asaoka & Yuki Watanabe

  2. First Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuokashimoaizuki, Eiheiji, Fukui, 910-1193, Japan

    Kazuhiro Itoh, Naoko Hosono, Tomoya Hirota, Takahiro Yamauchi & Hiromichi Iwasaki

  3. Department of Internal Medicine, National Hospital Organization Awara Hospital, Awara, Japan

    Kazuhiro Itoh

  4. Department of Infection Control and Prevention, University of Fukui Hospital, Fukui, Japan

    Kazuhiro Itoh & Hiromichi Iwasaki

  5. Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Fukui, Japan

    Masamichi Ikawa & Tomohisa Yamaguchi

  6. Division of Surgical Pathology, University of Fukui Hospital, Fukui, Japan

    Satomi Hatta & Yoshiaki Imamura

  7. Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan

    Ichizo Nishino

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  1. Kohei Asaoka
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Correspondence to Kazuhiro Itoh.

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All procedures used in this research were approved by the Ethical Committee of University of Fukui.

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Asaoka, K., Watanabe, Y., Itoh, K. et al. A case of eosinophilic fasciitis without skin manifestations: a case report in a patient with lupus and literature review. Clin Rheumatol 40, 2477–2483 (2021). https://doi.org/10.1007/s10067-020-05416-6

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  • DOI: https://doi.org/10.1007/s10067-020-05416-6

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