经过合适治疗后,大多数患者临床缓解。 尽管如此,老年人的死亡率很高,第一年内,死亡率估计在 6% 至 41% 之间。[44]Colbert RL, Allen DM, Eastwood D, et al. Mortality rate of bullous pemphigoid in a US medical center. J Invest Dermatol. 2004;122:1091-1095.http://www.ncbi.nlm.nih.gov/pubmed/15140208?tool=bestpractice.com[45]Bystryn JC, Rudolph JL. Why is the mortality of bullous pemphigoid greater in Europe than in the US? J Invest Dermatol. 2005;124:xx-xxi.http://www.ncbi.nlm.nih.gov/pubmed/15737186?tool=bestpractice.com
根据报告,在欧洲,开始治疗后 1 年,大疱性类天疱疮的死亡率(19% 至 41%)远高于美国(6% 至 12%)。[44]Colbert RL, Allen DM, Eastwood D, et al. Mortality rate of bullous pemphigoid in a US medical center. J Invest Dermatol. 2004;122:1091-1095.http://www.ncbi.nlm.nih.gov/pubmed/15140208?tool=bestpractice.com[45]Bystryn JC, Rudolph JL. Why is the mortality of bullous pemphigoid greater in Europe than in the US? J Invest Dermatol. 2005;124:xx-xxi.http://www.ncbi.nlm.nih.gov/pubmed/15737186?tool=bestpractice.com 此结局差异可能是由于治疗不同、种族差异、年龄差异、研究规模差异和患者选择偏倚。[21]Joly P, Benichou J, Lok C, et al. Prediction of survival for patients with bullous pemphigoid: a prospective study. Arch Dermatol. 2005;141:691-698.http://www.ncbi.nlm.nih.gov/pubmed/15967914?tool=bestpractice.com 在法国的 20 个皮肤病科开展了一项前瞻性、随机、对照试验,结果表明,只有年龄较大 (p=0.02) 和 Karnofsky 评分较低 (p<0.001) 能够独立预测死亡。[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[3]Walsh SR, Hogg D, Mydlarski PR. Bullous pemphigoid: From bench to bedside. Drugs. 2005;65:905-926.http://www.ncbi.nlm.nih.gov/pubmed/15892587?tool=bestpractice.com[21]Joly P, Benichou J, Lok C, et al. Prediction of survival for patients with bullous pemphigoid: a prospective study. Arch Dermatol. 2005;141:691-698.http://www.ncbi.nlm.nih.gov/pubmed/15967914?tool=bestpractice.com
Karnofsky 体能状态 (KPS) 评分
发现与大疱性类天疱疮直接相关的因素与患者预后都没有相关性。