Oliveira Luiza Barbosa, Maruta Celina Wakisaka, Miyamoto Denise, Salvadori Fernanda Aburesi, Santi Claudia Giuli, Aoki Valeria, Duarte-Neto Amaro Nunes
Autopsy Division - Anatomic Pathology Department - Faculty of Medicine - Universidade de São Paulo, São Paulo/SP - Brazil.
Department of Dermatology - Faculty of Medicine - Universidade de São Paulo, São Paulo/SP - Brazil.
Autops Case Rep. 2017 Mar 30;7(1):23-30. doi: 10.4322/acr.2017.008. eCollection 2017 Jan-Mar.
Pemphigus vulgaris is an autoimmune disease characterized by the formation of suprabasal intra-epidermal blisters on the skin and mucosal surfaces. Infectious diseases are the main cause of death in patients with pemphigus due to the disrupture of the physiological skin barrier, immune dysregulation, and the use of immunosuppressive medications leaving the patient prone to acquire opportunistic infections. We report the case of a 67-year-old woman diagnosed with pemphigus vulgaris, who was irregularly taking prednisone and mycophenolate mofetil. She was hospitalized because of a 1-month history of watery diarrhea and oral ulcers. Unfortunately, the patient died suddenly on the ward. The autopsy revealed a bilateral saddle pulmonary embolism, Gram-positive cocci bronchopneumonia, and gastrointestinal cytomegalovirus infection, causing extensive gastrointestinal mucosal ulcers.
寻常型天疱疮是一种自身免疫性疾病，其特征是在皮肤和黏膜表面形成表皮内基底上水泡。由于生理皮肤屏障破坏、免疫失调以及使用免疫抑制药物使患者易发生机会性感染，传染病是天疱疮患者的主要死亡原因。我们报告一例67岁女性，诊断为寻常型天疱疮，她不规则服用泼尼松和霉酚酸酯。她因1个月的水样腹泻和口腔溃疡病史入院。不幸的是，患者在病房突然死亡。尸检显示双侧鞍状肺栓塞、革兰氏阳性球菌支气管肺炎和胃肠道巨细胞病毒感染，导致广泛的胃肠道黏膜溃疡。
