Circulating plakin autoantibodies in a patient with erythema multiforme major: Are they pathogenic or a manifestation of epitope spreading?

Abstract

Erythema multiforme is a well-recognised entity but its pathogenesis remains elusive. Theories hypothesise a cell-mediated immune pathogenesis, however recent case reports have observed autoantibodies to the plakin family of proteins, suggesting a role for the humoral immune system. We present a case of erythema multiforme major with circulating desmoplakin autoantibodies in a 36-year old woman who was previously diagnosed with pemphigoid gestationis. The close correlation between the concentration of these autoantibodies and the severity of clinical disease strongly suggests a pathogenic role in her disease. As previous case reports have proposed, these autoantibodies may be directly pathogenic. Alternatively, the epiphenomenon of epitope spreading must be considered in the subset of patients with erythema multiforme major.

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

“…In our study, patients with EM‐PLK+ did not differ from classical features of EM major 1 . As previously reported, the most prevalent anti‐PLK‐Abs were anti‐DSP‐I and ‐II 6–11 . Nevertheless, we also found anti‐ENV and ‐PPL Abs and the association of the two or three types of anti‐PLK‐Abs in a subset of our patients 17 .…”

Under-reported finding in acral erythema multiforme

“…These erythematous areas are adjacent to the skin lesions of EM, so this nail change may be explained by the inflammatory process that cause vessel dilatation with an alteration of the blood supply in the nail bed. [ 7 ]…”

In vivo reflectance confocal microscopy of erythema multiforme and Stevens‐Johnson syndrome: a histopathological correlation based on a case series

“…It is believed that HSV-associated cases are caused by a T cell-mediated, cytotoxic immune response to keratinocytes containing the viral antigen HSV DNA polymerase gene ( pol ) [ 5 ] . A recent report also described a humoral immune response with circulating immune complexes [ 6 ] . SJS is more frequently triggered by drugs such as antiepileptic agents and sulfonamides, Clinically, it is characterized by disseminated, rather atypical, coalescing target lesions.…”