Naito Ryota, Hiwa Ryosuke, Inaba Ryuta, Murakami Kosaku, Kitoh Akihiko, Kaku Yo, Murata Teruasa, Ichimura Yuki, Okiyama Naoko, Nishino Ichizo, Shirakashi Mirei, Onizawa Hideo, Tsuji Hideaki, Kitagori Koji, Akizuki Shuji, Nakashima Ran, Onishi Akira, Tanaka Masao, Yoshifuji Hajime, Morinobu Akio
Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Center for Cancer Immunotherapy and Immunobiology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Mod Rheumatol Case Rep. 2025 Jan 16;9(1):84-87. doi: 10.1093/mrcr/rxae037.
Vesiculobullous dermatomyositis (VD) is a rare manifestation of dermatomyositis (DM) and has been suggested to be associated with malignancy. Although the myositis-specific autoantibodies are associated with distinct clinical presentations of DM, those associated with VD remain unclear. Here, we present the case of a 54-year-old man with VD who tested positive for antinuclear matrix protein 2 (NXP-2) antibodies, one of the DM-specific autoantibodies. Serological and histopathological findings did not support autoimmune blistering disease. Physical and histological findings suggested that the severe oedema in combination with the interface dermatitis of DM contributed to blister formation. Although a systemic examination was performed, no evidence of malignancy was found. Following initiation of immunosuppressive therapy, the patient showed significant improvement in both skin lesions and myositis. This case represents the first report of anti-NXP-2-positive VD without malignancy or autoimmune blistering disease. Subcutaneous oedema, a characteristic feature of anti-NXP-2-positive DM, could be related to the formation of VD.
水疱大疱性皮肌炎（VD）是皮肌炎（DM）的一种罕见表现，有人认为它与恶性肿瘤有关。尽管肌炎特异性自身抗体与DM的不同临床表现相关，但与VD相关的自身抗体仍不清楚。在此，我们报告一例54岁患有VD的男性，其抗核基质蛋白2（NXP - 2）抗体检测呈阳性，NXP - 2抗体是DM特异性自身抗体之一。血清学和组织病理学检查结果不支持自身免疫性水疱病。体格检查和组织学检查结果表明，严重水肿与DM的界面性皮炎共同导致了水疱形成。尽管进行了全身检查，但未发现恶性肿瘤的证据。在开始免疫抑制治疗后，患者的皮肤病变和肌炎均有显著改善。该病例是首例抗NXP - 2阳性且无恶性肿瘤或自身免疫性水疱病的VD报告。皮下水肿是抗NXP - 2阳性DM的一个特征性表现，可能与VD的形成有关。
