Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy

J Am Acad Dermatol. 2021 Jul;85(1):18-27. doi: 10.1016/j.jaad.2020.05.161. Epub 2021 Mar 5.

Abstract

Subepidermal (subepithelial) autoimmune blistering dermatoses are a group of rare skin disorders characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The fourth article in this continuing medical education series presents the current validated disease activity scoring systems, serologic parameters, treatments, and clinical trials for bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, bullous systemic lupus erythematosus, anti-p200 pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.

Keywords: anti-p200 pemphigoid; autoimmune blistering disease severity index system; autoimmune bullous disease quality of life; bullous pemphigoid; bullous pemphigoid disease area index; bullous systemic lupus erythematosus; dapsone; dermatitis herpetiformis; epidermolysis bullosa acquisita; linear IgA bullous dermatosis; mucous membrane pemphigoid; subepithelial autoimmune bullous dermatoses.

Publication types

  • Review

MeSH terms

  • Administration, Cutaneous
  • Administration, Oral
  • Autoantibodies / blood*
  • Autoantibodies / immunology
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy*
  • Autoimmune Diseases / immunology
  • Biomarkers / blood
  • Clinical Trials as Topic
  • Cytokines / blood
  • Cytokines / immunology
  • Dermis / immunology
  • Dermis / pathology
  • Drug Therapy, Combination / methods
  • Glucocorticoids / administration & dosage
  • Humans
  • Immunologic Factors / administration & dosage*
  • Photochemotherapy / methods*
  • Severity of Illness Index
  • Skin Diseases, Vesiculobullous / blood
  • Skin Diseases, Vesiculobullous / diagnosis
  • Skin Diseases, Vesiculobullous / drug therapy*
  • Skin Diseases, Vesiculobullous / immunology
  • Treatment Outcome

Substances

  • Autoantibodies
  • Biomarkers
  • Cytokines
  • Glucocorticoids
  • Immunologic Factors