Loczi-Storm Angela R, Iurillo Alyssa M, Mirza Fatima N, Clement Paige, Wisco Oliver J
College of Osteopathic Medicine - Northwest, Western University of Health Sciences, Lebanon, USA.
Dermatology, Indiana University School of Medicine, Indianapolis, USA.
Cureus. 2025 Aug 12;17(8):e89933. doi: 10.7759/cureus.89933. eCollection 2025 Aug.
Bullous pemphigoid (BP) is the most common autoimmune blistering disease, primarily affecting older adults and typically presenting with intensely pruritic, tense cutaneous bullae. While mucosal involvement is more common in other blistering disorders such as mucous membrane pemphigoid, it can occasionally occur in BP. This atypical presentation may confound diagnosis and cause treatment delays. Here, we report the case of a 67-year-old woman with idiopathic BP involving both cutaneous and oral mucosal surfaces. The disease began with a single bulla on the ear and a pruritic rash across the upper back, eventually progressing to widespread cutaneous bullae with painful gingival involvement. After a suboptimal response to prednisone and mycophenolate mofetil, remission was successfully achieved with rituximab. This case underscores the diagnostic complexity and treatment challenges often seen in BP with mucosal involvement. Early recognition of atypical variants and careful histopathologic assessment are essential for guiding effective therapy.
大疱性类天疱疮（BP）是最常见的自身免疫性水疱病，主要影响老年人，通常表现为剧烈瘙痒的紧张性皮肤水疱。虽然黏膜受累在其他水疱病如黏膜类天疱疮中更为常见，但在BP中偶尔也会发生。这种非典型表现可能会混淆诊断并导致治疗延误。在此，我们报告一例67岁女性特发性BP累及皮肤和口腔黏膜表面的病例。疾病始于耳部单个水疱和上背部瘙痒性皮疹，最终发展为广泛的皮肤水疱并伴有疼痛的牙龈受累。在对泼尼松和霉酚酸酯反应欠佳后，利妥昔单抗成功诱导缓解。该病例强调了BP伴黏膜受累时常见的诊断复杂性和治疗挑战。早期识别非典型变异型并进行仔细的组织病理学评估对于指导有效治疗至关重要。
