Department of Dermatology, University of Lübeck, Lübeck, Germany.
Center for Research on Inflammation of the Skin, University of Lübeck, Lübeck, Germany.
J Dermatol. 2021 Aug;48(8):1257-1261. doi: 10.1111/1346-8138.15912. Epub 2021 May 17.
Bullous pemphigoid (BP) is an autoimmune skin disease, caused by autoantibodies to BP180 and/or BP230. While both these autoantigens are expressed in the entire skin, only some parts of the body become affected. Rare clinical observations indicate that BP may also manifest locally, usually following exposure to triggers. Here, we evaluated the occurrence and potential triggers of localized BP (LBP) in a cohort of 285 BP patients. Medical records of all BP patients hospitalized between 2009 and 2019 were reviewed. In 7/285 BP patients, a localized variant was identified. In 5/7 LBP patients, the disease remained local, while in 2/7 patients, an initial LBP subsequently spread. All cases were preceded by presumptive triggers, including previously described triggers and bacterial infections. Overall, LBP is rare. LBP, however, might be underdiagnosed and should thus be considered in the differential diagnosis, particularly when trigger factors preceded.
大疱性类天疱疮(BP)是一种自身免疫性皮肤病,由针对 BP180 和/或 BP230 的自身抗体引起。虽然这两种自身抗原都在整个皮肤中表达,但只有身体的某些部位受到影响。罕见的临床观察表明,BP 也可能局部表现,通常在接触诱因后。在这里,我们评估了 285 例 BP 患者队列中局部性 BP(LBP)的发生和潜在诱因。回顾了 2009 年至 2019 年间住院的所有 BP 患者的病历。在 285 例 BP 患者中,有 7 例为局部性变异。在 7 例 LBP 患者中,5 例疾病仍局限于局部,而在 7 例患者中,2 例最初的 LBP 随后扩散。所有病例均有可疑诱因,包括先前描述的诱因和细菌感染。总的来说,LBP 是罕见的。然而,LBP 可能被漏诊,因此应在鉴别诊断中考虑,特别是在有诱因的情况下。