Messersmith Lynn, Krauland Kevin
Brooke Army Medical Center
Pemphigus is a group of vesiculobullous autoimmune diseases. The most common type of pemphigus is pemphigus vulgaris, characterized by mucocutaneous bullae. Among the many types of pemphigus, the rarest clinical variant is pemphigus vegetans. It distinguishes itself from pemphigus vulgaris by the formation of vegetative plaques in intertriginous areas and oral mucosa. Thought to be a subtype of pemphigus vulgaris, pemphigus vegetans represents 1-2% of all pemphigus . It is often difficult to diagnose and clinically misleading due to the variable presentation and presence of verrucous vegetations. There are two clinically recognized forms of pemphigus vegetans: the Hallopeau and Neumann. The Hallopeau type has an indolent course and characteristically demonstrates pustules that heal as vegetative plaques. The Neumann type is more severe and refractory to treatment, with vegetations that develop during an eruption of vesiculobullous lesions. The oral mucosa is usually involved. The most common cause of pemphigus vegetans is autoantibodies against desmoglein 3 (DSG3) and desmoglein 1 (DSG1). The mainstay of treatment for pemphigus vegetans is similar to that of pemphigus vulgaris, consisting of systemic corticosteroids and immunosuppressants.
天疱疮是一组水疱大疱性自身免疫性疾病。天疱疮最常见的类型是寻常型天疱疮，其特征为黏膜皮肤水疱。在众多天疱疮类型中，最罕见的临床变异型是增殖性天疱疮。它与寻常型天疱疮的区别在于在皮肤褶皱部位和口腔黏膜形成增殖性斑块。增殖性天疱疮被认为是寻常型天疱疮的一种亚型，占所有天疱疮病例的1 - 2%。由于其表现多样且存在疣状增殖，常常难以诊断且在临床上容易造成误导。增殖性天疱疮有两种临床公认的类型：哈洛波型和诺伊曼型。哈洛波型病程缓慢，其特征是脓疱愈合后形成增殖性斑块。诺伊曼型更为严重且治疗难治，在水疱大疱性皮损发作期间出现增殖。口腔黏膜通常会受累。增殖性天疱疮最常见的病因是针对桥粒芯糖蛋白3（DSG3）和桥粒芯糖蛋白1（DSG1）的自身抗体。增殖性天疱疮的主要治疗方法与寻常型天疱疮相似，包括全身用皮质类固醇和免疫抑制剂。
