A case of eosinophilic fasciitis without skin manifestations: a case report in a patient with lupus and literature revie
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CASE BASED REVIEW
A case of eosinophilic fasciitis without skin manifestations: a case report in a patient with lupus and literature review Kohei Asaoka 1 & Yuki Watanabe 1 & Kazuhiro Itoh 2,3,4 & Naoko Hosono 2 & Tomoya Hirota 2 & Masamichi Ikawa 5 & Tomohisa Yamaguchi 5 & Satomi Hatta 6 & Yoshiaki Imamura 6 & Ichizo Nishino 7 & Takahiro Yamauchi 2 & Hiromichi Iwasaki 2,4 Received: 16 June 2020 / Revised: 6 September 2020 / Accepted: 16 September 2020 # International League of Associations for Rheumatology (ILAR) 2020
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. Keywords Eosinophilic fasciitis . Diagnostic criteria . Skin manifestations . Systemic lupus erythematosus
Introduction Eosinophilic fasciitis (EF) is a rare connective tissue disease that was initially described by Shulman in 1974 [1]. There have since been approximately 400 cases of EF reported worldwide * Kazuhiro Itoh [email protected]
[2, 3]. EF is regarded as a fibrosing and sclerosing disorder that is associated with skin changes, such as edema, erythema, induration, peau d’orange appearance, and the groove sign, as the most characteristic manifestations of the disease [2–6]. According to previous findings, more than 95% of patients with EF show some of these skin manifestations [2, 3, 7–13]. Many patients also present with limb pain, joint contracture, muscle weakness, and a limited range of motion, which may be attributed to fascial inflammation and fibrosis [2, 6]. Characteristic laboratory findings for EF include peripheral eosinophilia, hypergammaglobulinemia, an elevated aldolase level, and increases in inflammatory markers, such as the erythrocyte sedimentation rate and C-reactive protein [2, 6]. Although the factors assumed to cause the disease include strenuous exercise, hemodialysis, borreliosis, and drugs, such as phenytoin, heparin, and nivolumab, the pathophysiological mechanisms underlying disease onset currently remain unclear [2]. We herein describe a Japanese case of EF without ski
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