University of Groningen, University Medical Center Groningen, Department of Dermatology, Center for Blistering Diseases, Groningen, The Netherlands.
University of Groningen, University Medical Center Groningen, Department of Dermatology, Center for Blistering Diseases, Groningen, The Netherlands.
J Am Acad Dermatol. 2018 May;78(5):989-995.e2. doi: 10.1016/j.jaad.2017.10.035. Epub 2017 Nov 1.
Bullous pemphigoid is an autoimmune disease that typically presents with tense bullae and severe pruritus. However, bullae can be lacking, a subtype termed nonbullous pemphigoid.
To summarize the reported characteristics of nonbullous pemphigoid.
The EMBASE and MEDLINE databases were searched using "nonbullous pemphigoid" and various synonyms. Case reports and series describing nonbullous pemphigoid were included.
The search identified 133 articles. After selection, 39 articles were included, presenting 132 cases. Erythematous, urticarial plaques (52.3%) and papules/nodules (20.5%) were the most reported clinical features. The mean age at presentation was 74.9 years. Histopathology was commonly nonspecific. Linear depositions of IgG and/or C3 along the basement membrane zone were found by direct immunofluorescence microscopy in 93.2%. Indirect immunofluorescence on salt-split skin was positive in 90.2%. The mean diagnostic delay was 22.6 months. A minority of patients (9.8%) developed bullae during the reported follow-up.
Results are mainly based on case reports and small case series.
Nonbullous pemphigoid is an underdiagnosed variant of pemphigoid that most often does not evolve to bullous lesions and mimics other pruritic skin diseases. Greater awareness among physicians is needed to avoid delay in diagnosis.
大疱性类天疱疮是一种自身免疫性疾病，通常表现为紧张性水疱和严重瘙痒。然而，也可能缺乏水疱，这是一种称为非大疱性类天疱疮的亚型。
总结非大疱性类天疱疮的报道特征。
使用“非大疱性类天疱疮”和各种同义词在 EMBASE 和 MEDLINE 数据库中进行搜索。纳入描述非大疱性类天疱疮的病例报告和系列。
搜索确定了 133 篇文章。经过选择，纳入了 39 篇文章，共介绍了 132 例病例。最常报告的临床特征是红斑性、荨麻疹样斑块（52.3%）和丘疹/结节（20.5%）。发病时的平均年龄为 74.9 岁。组织病理学通常是非特异性的。直接免疫荧光显微镜检查发现 93.2%的患者存在 IgG 和/或 C3 沿基底膜带线状沉积。盐裂皮肤间接免疫荧光检查阳性率为 90.2%。平均诊断延迟时间为 22.6 个月。在报告的随访中，少数患者（9.8%）出现水疱。
结果主要基于病例报告和小病例系列。
非大疱性类天疱疮是一种被低估的类天疱疮变体，通常不会发展为大疱性病变，且模仿其他瘙痒性皮肤病。医生需要提高认识，避免诊断延误。
