Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy -
Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy.
Ital J Dermatol Venerol. 2021 Aug;156(4):455-459. doi: 10.23736/S2784-8671.20.06562-1. Epub 2020 Jun 15.
Bullous pemphigoid (BP) is an autoimmune blistering disease caused by antibodies against the hemidesmosomal BP180 and/or BP230 proteins. There is an increasing evidence that the use of dipeptidyl peptidase-4 inhibitors, also known as gliptins, increases the risk for BP. The gliptins more frequently associated with BP are vildagliptin and sitagliptin. Clinical, immunological and pathological features of gliptin-associated BP have been reported to be distinct, compared to classic BP.
In this study, 15 gliptin-associated BP (g-BP) cases have been compared with 16 consecutive idiopathic BP (i-BP) to clarify whether g-BP has distinctive clinical and immunopathological characteristics. Comorbidities, concomitant treatments, latency of the onset of the disease and the time to achieve the remission were also considered.
The mean latency from drug intake to g-BP appearance was 9.4 months (median 10, inter-quartile range [IQR] 6-12). There were no differences in sex and age prevalence between the two groups (g-BP median age 77 years, IQR: 70-84; i-BP: 81 years, IQR: 72-86). There were no differences as far clinical presentation including disease severity, lesions types (urticarial and bullous) or mucosal involvement between g-BP and i-BP cases. The median antibody anti-BP180 and anti-BP230 titers was also similar between the two groups with 29.1 UI/mL (IQR: 12.9-65.3) and 11.8 UI/mL (IQR: 1.7-26.3), respectively. Gliptins were withdrawn in ten out of 15 patients and remission was achieved with systemic corticosteroids (0.3-0.7 mg/Kg daily) alone or in association with doxycycline (100-200 mg daily) within a mean of 8 months.
A non-inflammatory phenotype with less erythema, fewer urticarial lesions and fewer eosinophils in skin lesions has been associated with gliptins in selected Japanese BP populations. As reported in European studies, no significant differences among the considered variables in the g-BP and i-BP cases have been found in our study.
大疱性类天疱疮（BP）是一种由针对半桥粒 BP180 和/或 BP230 蛋白的抗体引起的自身免疫性水疱病。越来越多的证据表明，二肽基肽酶-4 抑制剂（也称为gliptins）的使用会增加 BP 的风险。与 BP 相关的 gliptins 更常与 vildagliptin 和 sitagliptin 相关。与经典 BP 相比，报道称 gliptin 相关 BP 的临床、免疫和病理特征明显不同。
在这项研究中，将 15 例 gliptin 相关 BP（g-BP）病例与 16 例连续特发性 BP（i-BP）病例进行比较，以明确 g-BP 是否具有独特的临床和免疫病理特征。还考虑了合并症、同时治疗、疾病发病潜伏期和达到缓解的时间。
从药物摄入到 g-BP 出现的平均潜伏期为 9.4 个月（中位数为 10，四分位距 [IQR] 为 6-12）。两组间的性别和年龄患病率无差异（g-BP 中位年龄为 77 岁，IQR：70-84；i-BP：81 岁，IQR：72-86）。g-BP 和 i-BP 病例的临床表现（疾病严重程度、皮损类型[荨麻疹样和大疱性]或粘膜受累）也无差异。两组间抗体抗 BP180 和抗 BP230 滴度也相似，分别为 29.1 UI/mL（IQR：12.9-65.3）和 11.8 UI/mL（IQR：1.7-26.3）。在 15 例患者中有 10 例停用了 gliptin，并用全身皮质类固醇（0.3-0.7 mg/Kg 每日）单独或联合多西环素（100-200 mg 每日）治疗，平均 8 个月内达到缓解。
在选定的日本 BP 人群中，与 gliptin 相关的非炎症表型具有较少的红斑、较少的荨麻疹样皮损和皮损中较少的嗜酸性粒细胞。与欧洲研究报道的情况一样，在我们的研究中，g-BP 和 i-BP 病例之间的考虑变量没有发现显著差异。
