Seltene Varianten der Pemphigoiderkrankungen

Translated title of the contribution: Rare variants of pemphigoid diseases

Kaan Yilmaz, Charlotte Kiehne, Maike M. Holtsche, Enno Schmidt*

*Corresponding author for this work

Abstract

Pemphigoid diseases comprise a heterogeneous group of subepidermal autoimmune blistering dermatoses characterized by autoantibodies against structural proteins of the dermal–epidermal junction. Recent decades have witnessed a significant surge in the incidence of these diseases, which, in addition to general aging of the population, can be attributed to the availability of precise diagnostic methods and improved knowledge of the clinical and immunopathological spectrum. While bullous pemphigoid, mucous membrane pemphigoid, and linear IgA disease account for most pemphigoid disorders, less frequent, presumably underdiagnosed variants are increasingly becoming relevant for clinicians. These include epidermolysis bullosa acquisita, anti-p200 pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, and recently defined entities such as IgM pemphigoid and Orf-induced pemphigoid. Accurate characterization and differentiation of these subtypes are not only of diagnostic relevance but may also be associated with therapeutic and prognostic implications for affected individuals. Due to the rarity of these diseases, no controlled prospective clinical trials currently exist, making their diagnosis and therapy challenging.

Translated title of the contributionRare variants of pemphigoid diseases
Original languageGerman
JournalDermatologie
Volume74
Issue number12
Pages (from-to)937-947
Number of pages11
ISSN2731-7005
DOIs
Publication statusPublished - 12.2022

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)
  • Centers: Center for Research on Inflammation of the Skin (CRIS)

DFG Research Classification Scheme

  • 204-05 Immunology
  • 205-19 Dermatology

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