Epidemiology of Pemphigus

Khalaf Kridin; Enno Schmidt

doi:10.1016/j.xjidi.2021.100004

Epidemiology of Pemphigus

JID Innov. 2021 Feb 20;1(1):100004. doi: 10.1016/j.xjidi.2021.100004. eCollection 2021 Mar.

Authors

Khalaf Kridin^{1

2}, Enno Schmidt^{1

3}

Affiliations

¹ Lűbeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
² The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel.
³ Department of Dermatology, University of Lübeck, Lübeck, Germany.

Abstract

Pemphigus is an epidemiologically heterogeneous group of autoimmune bullous diseases comprising pemphigus vulgaris (PV), pemphigus foliaceus, paraneoplastic pemphigus, IgA pemphigus, and pemphigus herpetiformis. Recently, our knowledge about the frequency of pemphigus, which is highly variable between different populations, has considerably expanded, and the first non-HLA genes associated with PV have been identified. In addition, a variety of comorbidities, including other autoimmune diseases, hematological malignancies, and psoriasis, have been described in this variant. Here, initial data about the impact of COVID-19 on this fragile patient population are discussed and perspectives for future epidemiological studies are outlined.

Keywords: ACE, angiotensin-converting enzyme; AIBD, autoimmune bullous disease; CAAR, chimeric autoantibody receptor; CI, confidence interval; DSG, desmoglein; EADV, European Academy of Dermatology and Venereology; EC, extracellular; EMA, European Medicines Agency; FS, fogo selvage; HR, hazard ratio; ICD, International Classification of Diseases; PF, pemphigus foliaceus; PNP, paraneoplastic pemphigus; PV, pemphigus vulgaris; SMR, standardized mortality ratio.

Publication types

Review