Taguchi Hirokazu, Ueda Yoshitaka, Shigehara Yohya, Yokogawa Naoto
Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Center, Fuchu, JPN.
Department of Dermatology, Tokyo Metropolitan Tama Medical Center, Fuchu, JPN.
Cureus. 2025 Feb 6;17(2):e78622. doi: 10.7759/cureus.78622. eCollection 2025 Feb.
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by cutaneous lesions such as heliotrope rash, Gottron's papules, and Gottron's sign. Panniculitis is rarely reported as a skin manifestation of DM. Herein, we present a case of anti-nuclear matrix protein 2 antibody (NXP-2)-positive DM relapse with panniculitis in the absence of muscle symptoms. A 32-year-old female patient was referred to our department. Nine years ago, she was diagnosed with DM and had received prednisolone (PSL) and tacrolimus therapy. However, she discontinued her visits four years ago. She most recently presented with erythematous lesions on the right thigh and buttocks without any muscle symptoms. Thirteen days later, she experienced upper limb weakness. Laboratory findings at her current presentation demonstrated elevated serum creatine kinase levels and positivity for anti-NXP-2 antibody. A skin biopsy of the erythema on her left thigh demonstrated findings consistent with healed panniculitis. Based on these findings, an anti-NXP-2 antibody-positive DM relapse was diagnosed. Her symptoms resolved with PSL and tacrolimus therapy. It is important to recognize that panniculitis is a significant, cutaneous manifestation of DM and can present as the initial manifestation of a DM relapse even in the absence of muscle symptoms.
皮肌炎（DM）是一种特发性炎性肌病，其特征为出现如向阳疹、Gottron丘疹和Gottron征等皮肤病变。脂膜炎作为DM的皮肤表现鲜有报道。在此，我们报告一例抗核基质蛋白2抗体（NXP-2）阳性的DM复发患者，该患者出现脂膜炎且无肌肉症状。一名32岁女性患者转诊至我科。9年前，她被诊断为DM，并接受了泼尼松龙（PSL）和他克莫司治疗。然而，4年前她停止了复诊。她最近一次就诊时右大腿和臀部出现红斑性病变，无任何肌肉症状。13天后，她出现上肢无力。此次就诊时的实验室检查结果显示血清肌酸激酶水平升高且抗NXP-2抗体呈阳性。对其左大腿红斑进行皮肤活检，结果符合愈合性脂膜炎。基于这些发现，诊断为抗NXP-2抗体阳性的DM复发。经PSL和他克莫司治疗后，她的症状得到缓解。认识到脂膜炎是DM的一种重要皮肤表现，即使在无肌肉症状的情况下也可作为DM复发的初始表现，这一点很重要。
