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Pattern-Specific Loss of Desmoplakin I and II Immunoreactivity in Erythema Multiforme and its Variants: A Possible

Vernon J Forrester1, Benjamin Tran2, Stephanie C Hein3

  • 1Department of Dermatology, University of Virginia, Charlottesville, VA.

The American Journal of Dermatopathology
|October 11, 2019
PubMed

Insights

Loss of desmoplakin (Dp) immunoreactivity in skin biopsies can help diagnose Erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). This finding offers a specific diagnostic marker for these severe mucocutaneous diseases.

Area of Science:

  • Dermatology
  • Immunopathology
  • Histopathology

Background:

  • Erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) are severe mucocutaneous diseases with high morbidity and mortality.
  • Autoantibodies to desmoplakin (Dp) I and II are implicated in EM, SJS, and TEN, leading to truncated Dp production and characteristic cellular changes.
  • These cellular changes include perinuclear clumping of keratin filaments and dyskeratotic keratinocytes observed under light microscopy.

Purpose of the Study:

  • To investigate whether absent or diminished desmoplakin (Dp) immunoreactivity in skin biopsies can serve as a diagnostic marker for EM, SJS, and TEN.
  • To determine the specificity of Dp immunostaining patterns in differentiating EM, SJS, and TEN from other interface dermatitides.

Main Methods:

  • Analyzed desmoplakin (Dp) immunostaining patterns in skin biopsy specimens from 20 patients diagnosed with EM or SJS/TEN.
  • Performed Dp immunostaining on specimens from patients with five other conditions that histologically mimic EM, SJS, and TEN.
  • Utilized a chi-squared (χ) test to statistically compare Dp staining patterns between the disease groups.

Main Results:

  • All skin biopsy samples from patients with EM, SJS, and TEN showed absent or markedly diminished desmoplakin (Dp) staining.
  • Dp immunostaining patterns were significantly different in EM, SJS, and TEN compared to the five investigated histologic mimics.
  • This study provides the first statistically significant evidence for the specificity of Dp staining patterns in diagnosing EM/SJS/TEN.

Conclusions:

  • Absent or diminished desmoplakin (Dp) immunoreactivity is a highly specific finding in skin biopsies of patients with EM, SJS, and TEN.
  • Dp immunostaining represents a valuable and specific diagnostic marker for differentiating EM, SJS, and TEN from other interface dermatitides.
  • This histopathological marker can aid in the accurate diagnosis of these severe mucocutaneous diseases.

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