Management of epidermolysis bullosa acquisita

Dermatol Clin. 2011 Oct;29(4):643-7. doi: 10.1016/j.det.2011.06.020. Epub 2011 Aug 15.

Abstract

Epidermolysis bullosa acquisita is a rare and debilitating autoimmune mucocutaneous blistering disease caused by autoantibodies directed against type VII collagen or anchoring fibrils in the subepidermal basement membrane zone. Treatment is quite challenging because this disease can be recalcitrant to multiple modalities. This article discusses the current management of this disease.

Publication types

  • Review

MeSH terms

  • Antibodies, Monoclonal, Murine-Derived / therapeutic use
  • Autoantibodies / immunology
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology
  • Chronic Disease
  • Colchicine / therapeutic use
  • Collagen Type VII / immunology
  • Cyclosporine / therapeutic use
  • Dapsone / therapeutic use
  • Dermatologic Agents / therapeutic use
  • Epidermolysis Bullosa Acquisita / drug therapy*
  • Epidermolysis Bullosa Acquisita / immunology
  • Epidermolysis Bullosa Acquisita / therapy
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Mycophenolic Acid / analogs & derivatives
  • Mycophenolic Acid / therapeutic use
  • Photochemotherapy
  • Plasmapheresis
  • Rituximab
  • Sulfapyridine / therapeutic use
  • Sulfasalazine / therapeutic use

Substances

  • Antibodies, Monoclonal, Murine-Derived
  • Autoantibodies
  • Collagen Type VII
  • Dermatologic Agents
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Sulfasalazine
  • Rituximab
  • Cyclosporine
  • Dapsone
  • Mycophenolic Acid
  • Colchicine
  • Sulfapyridine