Linear immunoglobulin A bullous dermatosis

Clin Dermatol. 2012 Jan-Feb;30(1):38-50. doi: 10.1016/j.clindermatol.2011.03.008.

Abstract

Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is an autoimmune mucocutaneous disorder characterized by subepithelial bullae, with IgA autoantibodies directed against several different antigens in the basement membrane zone. Its immunopathologic characteristic resides in the presence of a continuous linear IgA deposit along the basement membrane zone, which is clearly visible on direct immunofluorescence. This disorder shows different clinical features and distribution when adult-onset of linear IgA disease is compared with childhood-onset. Diagnosis is achieved via clinical, histopathologic, and immunopathologic examinations. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time.

Publication types

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

MeSH terms

  • Adult
  • Age of Onset
  • Anti-Infective Agents / therapeutic use
  • Autoantibodies / analysis*
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology
  • Basement Membrane / immunology
  • Child
  • Fluorescent Antibody Technique, Direct
  • Humans
  • Immunoglobulin A / immunology*
  • Skin Diseases, Vesiculobullous / diagnosis*
  • Skin Diseases, Vesiculobullous / drug therapy
  • Skin Diseases, Vesiculobullous / immunology*
  • Skin Diseases, Vesiculobullous / pathology

Substances

  • Anti-Infective Agents
  • Autoantibodies
  • Immunoglobulin A