Epidemiological and clinical characteristics of patients with pemphigus treated in Białystok in 2001-2018.

BACKGROUND

Pemphigus is a group of potentially fatal dermatological autoimmune disorders.

AIM

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.

MATERIAL AND METHODS

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.

RESULTS

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.

CONCLUSIONS

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.