Patsatsi Aikaterini, Kyriakou Aikaterini, Werth Victoria P
Autoimmune Bullous Diseases Outpatient Unit, 2nd Department of Dermatology, Aristotle University School of Medicine, Thessaloniki, Greece.
Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.
Pediatr Dermatol. 2019 Mar;36(2):184-188. doi: 10.1111/pde.13717. Epub 2018 Dec 19.
Bullous pemphigoid (BP) is the most common autoimmune blistering disease affecting the elderly but is quite rare in childhood. The majority of pediatric cases have been reported during early childhood. Adolescence is divided into three phases: early (10-13 years), middle (14-17), and late (18-21). This review aimed to identify BP cases in adolescence and demonstrate their clinical features and course. Our literature search was performed in Medline with the terms "bullous pemphigoid in childhood and adolescence," "childhood bullous pemphigoid," "juvenile bullous pemphigoid," and "autoimmune blistering and autoimmune bullous diseases in childhood." The data extraction for late adolescence was limited by the fact that this age group is included in adult BP registries. We identified nine cases in early adolescence. Mucosa were affected in 5 of 9 cases. Treatment consisted of systemic prednisone (8/9), in combination with dapsone (2/9), azathioprine (2/9), or erythromycin/nicotinamide (1/9). Relapses were reported in 3 of 9 cases. We identified five cases occuring in middle adolescence. Mucosa were not affected. Treatment consisted of systemic prednisone (5/5), in combination with dapsone (3/5), azathioprine (2/5), doxycycline/nicotinamide (1/5), or mycophenolate mofetil (1/5). Relapses were reported in two of five cases. No case of BP in the late adolescence was included in the results, as only one case met the search criteria, and overlapped with pemphigus vulgaris. With only 14 cases found in our review, BP in adolescence appears even rarer than in earlier childhood. Despite its low prevalence, BP should be included in the differential diagnosis of autoimmune blistering diseases in adolescents.
大疱性类天疱疮（BP）是影响老年人的最常见自身免疫性水疱病，但在儿童期相当罕见。大多数儿科病例报告于幼儿期。青春期分为三个阶段：早期（10 - 13岁）、中期（14 - 17岁）和晚期（18 - 21岁）。本综述旨在识别青春期的BP病例，并展示其临床特征和病程。我们在Medline上进行文献检索，检索词为“儿童和青少年期大疱性类天疱疮”“儿童大疱性类天疱疮”“青少年大疱性类天疱疮”以及“儿童自身免疫性水疱病和自身免疫性大疱性疾病”。由于该年龄组被纳入成人BP登记系统，晚期青春期的数据提取受到限制。我们在青春期早期识别出9例病例。9例中有5例黏膜受累。治疗包括全身使用泼尼松（9例中的8例），联合氨苯砜（9例中的2例）、硫唑嘌呤（9例中的2例）或红霉素/烟酰胺（9例中的1例）。9例中有3例报告有复发。我们在青春期中期识别出5例病例。黏膜未受累。治疗包括全身使用泼尼松（5例中的5例），联合氨苯砜（5例中的3例）、硫唑嘌呤（5例中的2例）、强力霉素/烟酰胺（5例中的1例）或霉酚酸酯（5例中的1例）。5例中有2例报告有复发。晚期青春期没有BP病例纳入结果，因为只有1例符合检索标准，且与寻常型天疱疮重叠。在我们的综述中仅发现14例病例，青春期的BP似乎比幼儿期更为罕见。尽管其患病率较低，但BP应纳入青少年自身免疫性水疱病的鉴别诊断中。
