Gruber-Wackernagel Alexandra, Schug Tanja, Graier Thomas, Legat Franz J, Rinner Hanna, Hofer Angelika, Quehenberger Franz, Wolf Peter
Research Unit for Photodermatology, Department of Dermatology, Medical University of Graz, Graz, Austria.
Institute for Medical Informatics, Statistics and Documentation, Medical University of Graz, Graz, Austria.
Front Med (Lausanne). 2021 Jul 16;8:694281. doi: 10.3389/fmed.2021.694281. eCollection 2021.
Little is known about the long-term course of polymorphic light eruption (PLE). To predict disease course, a questionnaire was sent to patients whose PLE had been diagnosed between March 1990 and December 2018 and documented in the Austrian Cooperative Registry for Photodermatoses. In January 2019, 205 PLE patients were contacted by mail and asked to complete a questionnaire on their disease course, including whether the skin's sun sensitivity had normalized (i.e., PLE symptoms had disappeared), improved, stayed the same, or worsened over time. Patients who reported normalization of sun sensitivity were asked to report when it had occurred. Ninety-seven patients (79 females, 18 males) returned a completed questionnaire. The mean (range) duration of follow-up from PLE onset was 29.6 (17-54) years for females and 29.4 (16-47) years for males. The disease disappeared in 32 (41%) females after 17.4 (2-41) years and in 4 (24%) males after 11.8 (5-26) years. Twenty-nine (37%) females and 6 (35%) males reported improvement of symptoms over time; 15 females (19%) and 7 males (41%) reported no change; and 3 females (4%) and no males reported worsening of symptoms. Kaplan-Meier analysis revealed that after 20 years 74% (95%CI, 64-82%) of patients still suffered from PLE. PLE lesion persistence (>1 week) tended to predict a prolonged course of PLE. PLE usually takes a long-term course over many years though in most patients its symptoms improve or disappear over time. How improvement relates to the pathophysiology of the disease remains to be determined.
关于多形性日光疹(PLE)的长期病程,人们所知甚少。为了预测疾病进程,我们向1990年3月至2018年12月期间被诊断为PLE且其病例记录在奥地利光皮肤病合作登记处的患者发送了一份问卷。2019年1月,我们通过邮件联系了205名PLE患者,要求他们填写一份关于疾病进程的问卷,包括皮肤对阳光的敏感性是否已恢复正常(即PLE症状是否已消失)、是否有所改善、保持不变或随时间恶化。报告阳光敏感性恢复正常的患者被要求报告其发生时间。97名患者(79名女性,18名男性)返回了完整的问卷。女性从PLE发病开始的平均(范围)随访时间为29.6(17 - 54)年,男性为29.4(16 - 47)年。17.4(2 - 41)年后,32名(41%)女性的疾病消失,11.8(5 - 26)年后,4名(24%)男性的疾病消失。29名(37%)女性和6名(35%)男性报告症状随时间有所改善;15名女性(19%)和7名男性(41%)报告无变化;3名女性(4%)报告症状恶化,男性无人报告症状恶化。Kaplan - Meier分析显示,20年后74%(95%CI,64 - 82%)的患者仍患有PLE。PLE皮损持续存在(>1周)往往预示着PLE病程延长。PLE通常会经历多年的长期病程,不过大多数患者的症状会随时间改善或消失。改善与疾病病理生理学之间的关系仍有待确定。