Bullous pemphigoid: from the clinic to the bench

Clin Dermatol. 2012 Jan-Feb;30(1):3-16. doi: 10.1016/j.clindermatol.2011.03.005.

Abstract

Bullous pemphigoid (BP) constitutes the most frequent autoimmune subepidermal blistering disease. It is associated with autoantibodies directed against the BP antigens 180 (BP180, BPAG2) and BP230 (BPAG1-e). The pathogenicity of anti-BP180 antibodies has been convincingly demonstrated in animal models. The clinical features of BP are extremely polymorphous. The diagnosis of BP critically relies on immunopathologic findings. The recent development of novel enzyme-linked immunosorbent assays has allowed the detection of circulating autoantibodies with relatively high sensitivity and specificity. Although potent topical steroids have emerged in the past decade as first-line treatment of BP, management of the disease may be challenging.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Administration, Topical
  • Animals
  • Autoantibodies / blood*
  • Autoantigens / blood*
  • Carrier Proteins / blood*
  • Cytoskeletal Proteins / blood*
  • Dermatologic Agents / administration & dosage
  • Dystonin
  • Enzyme-Linked Immunosorbent Assay
  • Glucocorticoids / administration & dosage
  • Humans
  • Nerve Tissue Proteins / blood*
  • Non-Fibrillar Collagens / blood*
  • Pemphigoid, Bullous / drug therapy
  • Pemphigoid, Bullous / immunology*
  • Pemphigoid, Bullous / pathology*
  • Secondary Prevention
  • Severity of Illness Index
  • Skin / pathology

Substances

  • Autoantibodies
  • Autoantigens
  • Carrier Proteins
  • Cytoskeletal Proteins
  • DST protein, human
  • Dermatologic Agents
  • Dystonin
  • Glucocorticoids
  • Nerve Tissue Proteins
  • Non-Fibrillar Collagens
  • collagen type XVII