Medical University of Bialystok Department of Dermatology and Venereology.
Przegl Epidemiol. 2020;74(3):457-465. doi: 10.32394/pe.74.39.
Pemphigus is a group of potentially fatal dermatological autoimmune disorders.
Analysis of cases of pemphigus vulgaris (PV) and pemphigus foliaceus (PF) newly diagnosed and treated at the Department of Dermatology and Venereology, Medical University of Białystok, North-east Poland in years 2001-2018.
A retrospective analysis and comparison of sociodemographic, epidemiological and clinical characteristics of PV and PF patients, including: age, gender, residency, initial severity of skin lesions, involvement of mucous membranes, co-morbidities and their treatment, efficacy of therapy.
Sixty-two new cases - 41 (66.13%) of PV and 21 (33.87%) of PF were diagnosed. The average age of PV patients was 54.85±12.35 years and those of PF - 63.81±31.52 years, P<0.05. Females constituted 75.61% and 61.90% in PV and PF group, respectively. Majority of patients with PV were residents of urban and these with PF - of rural areas (70.73% and 66.67%, respectively). On admission, 14 patients with PV (34.15%) and 11 with PF (52.38%) had more than 30% of body surface area involved. In 22 (53.66%) PV mucous membrane (oropharyngeal cavity) was involved. Ten (24.39%) patients with PV and 12 (57.14%) - with PF had more than one concomitant disorder (P<0.05). In treatment prednisone in monotherapy or with additional immunosuppressive agent was mainly used. The disease relapsed within three years after achieving clinical and immunological remission in 29.27% of PV and in 38.10% of PF patients.
PF patients are older than PV ones, more frequently live in rural areas, have more comorbidities. Females constitute majority of pemphigus patients. The disease may relapse in about one third of patients. Because of frequent comorbidities, also these related to pemphigus treatment, patients with pemphigus require complex and multispecialistic medical care.
天疱疮是一组潜在致命的皮肤科自身免疫性疾病。
分析 2001 年至 2018 年期间在波兰东北部比亚韦斯托克医科大学皮肤病学和性病科新诊断和治疗的寻常型天疱疮（PV）和落叶型天疱疮（PF）病例。
回顾性分析和比较 PV 和 PF 患者的社会人口统计学、流行病学和临床特征，包括：年龄、性别、居住地、皮肤损伤初始严重程度、黏膜受累、合并症及其治疗、治疗效果。
共诊断出 62 例新病例——41 例（66.13%）PV 和 21 例（33.87%）PF。PV 患者的平均年龄为 54.85±12.35 岁，PF 患者为 63.81±31.52 岁，P<0.05。女性在 PV 和 PF 组中分别占 75.61%和 61.90%。PV 患者主要居住在城市，而 PF 患者主要居住在农村（分别为 70.73%和 66.67%）。入院时，14 例 PV（34.15%）和 11 例 PF（52.38%）患者的体表面积受累超过 30%。22 例（53.66%）PV 患者有口腔黏膜受累。10 例（24.39%）PV 患者和 12 例（57.14%）PF 患者有超过一种合并症（P<0.05）。在治疗中，主要使用泼尼松单药或联合免疫抑制剂。在达到临床和免疫缓解后三年内，29.27%的 PV 患者和 38.10%的 PF 患者疾病复发。
PF 患者比 PV 患者年龄大，更多地居住在农村地区，合并症更多。女性构成了天疱疮患者的大多数。大约三分之一的患者可能会复发。由于频繁的合并症，包括与天疱疮治疗相关的合并症，天疱疮患者需要复杂和多学科的医疗护理。
