Pemphigus is a group of autoimmune blistering disease caused by autoantibodies against desmoglein 1 and 3. The two major subtypes of pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Their epidemiology is known to differ by ethnicities. Hence, using a nationwide inpatient database in Japan, we retrospectively identified 3795 patients admitted with either PV (2608 patients) or PF (1187 patients) and investigated their backgrounds, treatments, and outcomes. Overall, the median age at admission was 65 (interquartile range, 51–76) years. Patients with PV were younger (median, 62 vs 72 years, P<0.001) and had fewer comorbidities such as diabetes mellitus, hypertension, and chronic heart failure than those with PF. Nearly half of the patients were treated with intensive treatments approved in Japan (i.e., steroid pulse treatment, intravenous immunoglobulin, or plasmapheresis). The proportions of patients receiving the intensive treatments were higher for patients with PV than those with PF (steroid pulse treatment [13.3% vs 9.6%, P=0.003], intravenous immunoglobulin [38.3% vs 33.4%, P=0.004], and plasmapheresis [9.3% vs 7.4%, P=0.054]). Patients with PV showed lower in-hospital mortality (2.1% vs 4.0%, P=0.001) but longer length of stay and higher hospitalization costs than those with PF. This large-scale retrospective cohort study provides information on inpatients with pemphigus in one of the most aging societies in the world.

Pemphigus is a group of autoimmune blistering disease caused by autoantibodies against desmoglein 1 and 3. The two major subtypes of pemphigus are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Their epidemiology is known to differ by ethnicities. Hence, using a nationwide inpatient database in Japan, we retrospectively identified 3784 patients admitted with either PV (2598 patients) or PF (1186 patients) and investigated their backgrounds, treatments, and outcomes. Overall, the median age at admission was 65 (interquartile range, 51–76) years. Patients with PV were younger (median, 62 vs 72 years, P<0.001) and had fewer comorbidities such as diabetes mellitus, hypertension, and chronic heart failure than those with PF. Nearly half of the patients were treated with intensive treatments approved in Japan (i.e., steroid pulse treatment, intravenous immunoglobulin, or plasmapheresis). The proportions of patients receiving the intensive treatments were higher for patients with PV than those with PF (steroid pulse treatment [16% vs 12%, P=0.003], intravenous immunoglobulin [38% vs 33%, P=0.004], and plasmapheresis [9.4% vs 7.4%, P=0.051]). Patients with PV showed lower in-hospital mortality (2.2% vs 4.0%, P=0.002) but longer length of stay and higher hospitalization costs than those with PF. This large-scale retrospective cohort study provides information on inpatients with pemphigus in one of the most aging societies in the world.

ESDR084 - Clinical characteristics and treatments of pemphigus vulgaris and pemphigus foliaceus: a descriptive study of 3795 patients using a nationwide inpatient database in Japan

The scientific meeting of the European Society for Dermatological Research (ESDR) is held yearly, usually in September. With an international attendance of more than 1000 skin scientists from around the globe, the four-day event includes short oral presentations from peer-reviewed abstracts, keynote lectures from international experts, sponsored symposia, satellite programs, educational events as well as an important focus on posters, networking and building collaborations amongst scientists. For more information about this year’s meeting visit our website.