Hyobu Rie, Mori Miho, Maeda Tatsuo, Fujimori Kazuki, Shimai Yukako, Naito Makiko, Masuda Masayuki, Ohira Tatsuo, Ikeda Norihiko, Okubo Yukari, Harada Kazutoshi
Department of Dermatology, Tokyo Medical University, Tokyo, Japan.
Department of Neurology, Tokyo Medical University, Tokyo, Japan.
J Dermatol. 2024 Dec;51(12):1674-1678. doi: 10.1111/1346-8138.17519. Epub 2024 Oct 26.
Thymoma-associated multiorgan autoimmunity (TAMA) presents with skin symptoms similar to those of graft-versus-host disease (GVHD), liver dysfunction, and enteritis, in the absence of a history of hematopoietic stem cell or bone marrow transplantation. TAMA is a type of paraneoplastic syndrome associated with thymoma. Its etiology is unclear but is thought to be a result of breakdown of immune tolerance. Histopathologically, TAMA is characterized by epidermal acanthosis with parakeratosis, individual cell keratinization, liquefaction degeneration, and intraepidermal infiltration of CD8-positive lymphocytes. A 64-year-old female patient with a history of myasthenia gravis and thymoma treated with prednisolone (10 mg/day) and cyclosporine (150 mg/day) experienced erythema on her trunk after coronavirus disease 2019 (COVID-19) onset. A psoriatic drug eruption was suspected and the possible causative drug was discontinued, but the skin rash failed to improve. A skin biopsy demonstrated GVHD-like histopathological findings. Diarrhea, abdominal pain, and duodenal perforation occurred concurrently, leading to the diagnosis of TAMA. Thereafter, the patient continued prednisolone and cyclosporine in the same doses as the TAMA treatment and added topical steroids. During the disease course, candida fungemia and cytomegalovirus infection developed, resulting in the patient's death. The TAMA was considered to have been caused by the release of inflammatory cytokines, autoreactive T cell activation, and regulatory T cell dysfunction induced by COVID-19.
胸腺瘤相关多器官自身免疫(TAMA)表现为皮肤症状类似于移植物抗宿主病(GVHD)、肝功能障碍和肠炎,且无造血干细胞或骨髓移植病史。TAMA是一种与胸腺瘤相关的副肿瘤综合征。其病因尚不清楚,但被认为是免疫耐受破坏的结果。组织病理学上,TAMA的特征为表皮棘层肥厚伴角化不全、个别细胞角化、液化变性以及CD8阳性淋巴细胞的表皮内浸润。一名64岁女性患者,有重症肌无力和胸腺瘤病史,正在接受泼尼松龙(10毫克/天)和环孢素(150毫克/天)治疗,在2019冠状病毒病(COVID-19)发病后躯干出现红斑。怀疑是银屑病样药疹,停用了可能的致病药物,但皮疹未改善。皮肤活检显示出类似GVHD的组织病理学表现。同时出现腹泻、腹痛和十二指肠穿孔,从而诊断为TAMA。此后,患者继续使用与TAMA治疗相同剂量的泼尼松龙和环孢素,并加用局部类固醇。在病程中,发生了念珠菌血症和巨细胞病毒感染,导致患者死亡。TAMA被认为是由COVID-19诱导的炎性细胞因子释放、自身反应性T细胞活化和调节性T细胞功能障碍引起的。