Porro Adriana Maria, Seque Camila Arai, Ferreira Maria Carolina Corsi, Enokihara Milvia Maria Simões E Silva
Department of Dermatology, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Department of Pathology, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
An Bras Dermatol. 2019 Jul 29;94(3):264-278. doi: 10.1590/abd1806-4841.20199011.
Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.
寻常型天疱疮是一种慢性自身免疫性大疱性皮肤病，由针对桥粒芯糖蛋白1和3产生自身抗体所致。它是天疱疮最常见、最严重的类型，全球均有发生，通常发病年龄在40至60岁之间。通常始于口腔黏膜的水疱和糜烂，随后出现其他黏膜损害以及皮肤松弛水疱，这些水疱可呈播散性。有一种临床变型，即增殖型天疱疮，其特征是在皮肤大褶皱处出现增殖性损害。临床怀疑可通过细胞学检查、组织病理学检查以及直接和间接免疫荧光试验来确诊。治疗采用全身性皮质类固醇，可能会联合使用免疫抑制药物，其中包括硫唑嘌呤和霉酚酸酯。更严重的病例可能从静脉脉冲疗法形式的皮质类固醇中获益，最近的研究表明抗CD20免疫生物药物利妥昔单抗具有有益效果。这是一种慢性病，死亡率约为10%，败血症是主要死因。患者需要长期的多学科随访。
