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Erythema multiforme associated with anti-plakin antibodies: a multicentric retrospective case series

A Weill1,2,3, V Descamps4, F Chasset5

  • 1Department of Dermatology, CHU Henri Mondor, AP-HP, Créteil, France.

Journal of the European Academy of Dermatology and Venereology : JEADV
|May 24, 2022
PubMed
Abstract

Insights

Erythema multiforme with auto-antibodies against plakins (EM-PLK+) is often severe and recurrent. These difficult-to-treat cases highlight the need for further research into anti-PLK-Abs in EM.

Area of Science:

  • Immunodermatology
  • Autoimmunity
  • Mucocutaneous diseases

Background:

  • Erythema multiforme (EM) is an inflammatory condition often triggered by herpes simplex virus (HSV).
  • A subset of EM patients exhibit auto-antibodies against plakins (anti-PLK-Abs), termed EM-PLK+.
  • Clinical and immunological characteristics of EM-PLK+ remain poorly understood.

Purpose of the Study:

  • To delineate the clinical and immunological features of EM-PLK+.
  • To evaluate the treatment response in EM-PLK+ patients.
  • To investigate the association between EM and anti-PLK-Abs.

Main Methods:

  • Retrospective multicentric study (January 2009 - December 2020).
  • Selection of EM-PLK+ patients from Bichat hospital's immunological laboratory database.
  • Detection of anti-PLK-Abs via immunofluorescence assay, immunoblotting, and/or ELISA; exclusion of alternative diagnoses.

Main Results:

  • 29 EM-PLK+ patients included (83% major EM, 79% relapsing EM).
  • Typical lesions observed: targetoid (54%) and target-like (38%) cutaneous lesions; frequent oral (96%) and genital (68%) mucosal involvement.
  • Desmoplakin-I/II Abs were most common (69%); 57% of relapsing cases remained active despite treatment; antivirals were ineffective in preventing EM-HSV relapses (94%).

Conclusions:

  • The role of anti-PLK-Abs in EM requires further elucidation.
  • Systematic research into anti-PLK-Abs may clarify their association with EM pathogenesis (humoral immunity vs. epitope spreading).
  • EM-PLK+ is associated with severe, relapsing, and treatment-resistant forms of EM.

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