Epidermolysis bullosa acquisita

Clin Dermatol. 2012 Jan-Feb;30(1):60-9. doi: 10.1016/j.clindermatol.2011.03.011.

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen (C7) structures, a major component of anchoring fibrils, which attach the epidermis to the dermis. EBA patients have tissue-bound and circulating antitype C7 autoantibodies that attack type C7 and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These immunoglobulin G antitype C7 antibodies are pathogenic, because when they are injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmapheresis, photopheresis, infliximab, and intravenous immunoglobulin.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Animals
  • Autoantibodies / immunology*
  • Collagen Type VII / immunology
  • Cyclosporine / therapeutic use
  • Dapsone / therapeutic use
  • Enzyme-Linked Immunosorbent Assay
  • Epidermolysis Bullosa Acquisita / classification
  • Epidermolysis Bullosa Acquisita / diagnosis*
  • Epidermolysis Bullosa Acquisita / drug therapy*
  • Epidermolysis Bullosa Acquisita / immunology
  • Epidermolysis Bullosa Acquisita / pathology
  • Humans
  • Immunoblotting
  • Immunoglobulin G / immunology
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Rare Diseases
  • Severity of Illness Index
  • Skin / immunology
  • Skin / pathology

Substances

  • Adrenal Cortex Hormones
  • Autoantibodies
  • Collagen Type VII
  • Immunoglobulin G
  • Immunologic Factors
  • Immunosuppressive Agents
  • Cyclosporine
  • Dapsone