根据报告,美国的大疱性类天疱疮患病率为每百万人群 6 至 10 例,平均发作年龄为 65 岁。[2]Yeh SW, Ahmed B, Sami N, et al. Blistering disorders: diagnosis and treatment. Dermatol Ther. 2003;16:214-223.http://www.ncbi.nlm.nih.gov/pubmed/14510878?tool=bestpractice.com[5]Korman NJ. Bullous pemphigoid. The latest in diagnosis, prognosis, and therapy. Arch Dermatol. 1998;134:1137-1141.http://www.ncbi.nlm.nih.gov/pubmed/9762028?tool=bestpractice.com 该疾病主要累及老年人,平均发病年龄为 80-89 岁;有人担心,随着高龄人群的增多,发病率可能增加。[6]Jung M, Kippes W, Messer G, et al. Increased risk of bullous pemphigoid in male and very old patients: A population-based study on incidence. J Am Acad Dermatol. 1999;41:266-268.http://www.ncbi.nlm.nih.gov/pubmed/10426901?tool=bestpractice.com 男性受累频率高于女性。[2]Yeh SW, Ahmed B, Sami N, et al. Blistering disorders: diagnosis and treatment. Dermatol Ther. 2003;16:214-223.http://www.ncbi.nlm.nih.gov/pubmed/14510878?tool=bestpractice.com[6]Jung M, Kippes W, Messer G, et al. Increased risk of bullous pemphigoid in male and very old patients: A population-based study on incidence. J Am Acad Dermatol. 1999;41:266-268.http://www.ncbi.nlm.nih.gov/pubmed/10426901?tool=bestpractice.com 罕见情况下,该疾病见于儿童。[7]Chimanovitch I, Hamm H, Georgi M, et al. Bullous pemphigoid of childhood: Autoantibodies target the same epitopes within the NC16A domain of BP180 as autoantibodies in bullous pemphigoid of adulthood. Arch Dermatol. 2000;136:527-532.http://www.ncbi.nlm.nih.gov/pubmed/10768652?tool=bestpractice.com[8]Fisler RE, Saeb M, Liang MG, et al. Childhood bullous pemphigoid: a clinicopathologic study and review of the literature. Am J Dermatopathol. 2003;25:183-189.http://www.ncbi.nlm.nih.gov/pubmed/12775979?tool=bestpractice.com
一项为期 2 年的回顾性研究调查了在新加坡全国皮肤中心见到的所有表皮下免疫性大疱病,发现大疱性类天疱疮占所有诊断的 88%,好发于华裔,平均发作年龄为 77 岁。[9]Wong SN, Chua SH. Spectrum of subepidermal immunobullous disorders seen at the national skin centre, Singapore: a 2-year review. Br J Dermatol. 2002;147:476-480.http://www.ncbi.nlm.nih.gov/pubmed/12207586?tool=bestpractice.com 在苏格兰东北部,大疱性类天疱疮的发病率(每年每百万人群 14 例)高于欧洲大陆。[10]Gudi VS, White MI, Cruickshank N, et al. Annual incidence and mortality of bullous pemphigoid in the Grampian region of north-east Scotland. Br J Dermatol. 2005;153:424-427.http://www.ncbi.nlm.nih.gov/pubmed/16086760?tool=bestpractice.com 这个差异的原因尚不清楚,可能与不同人种对自身免疫性皮肤病的易感性、所研究人群的易感因素 HLA 基因型或环境影响有关。