Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype

Int J Dermatol. 2004 Jun;43(6):443-6. doi: 10.1111/j.1365-4632.2004.02016.x.

Abstract

Background: Autoantibodies in linear immunoglobulin A (IgA) disease (LAD) are reported to be of IgA class and directed against a 97-120 kDa epidermal antigen.

Methods: We report a 39-year-old woman with clinical features of LAD and with circulating IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180).

Results: Histopathology of lesional skin revealed a subepidermal blister with mixed inflammatory cell infiltrate. Direct immunofluorescence of perilesional skin showed linear deposits of IgA along the dermal-epidermal junction. The antigen specificity of the patient's circulating antibodies was determined by Western blotting and enzyme-linked immunoabsorbent assay (ELISA) using various antigen sources, including cultured human keratinocytes, dermal protein lysates, and purified laminin-5, as well as proteins corresponding to BP180, the 230 kDa bullous pemphigoid antigen (BP230), laminin-5 subunits, and collagen IV alpha1-alpha6 chains. IgA and IgG antibodies in the patient's serum were directed against BP180, and no IgA or IgG reactivity was found against the other skin antigens.

Conclusions: These data provide evidence for the presence of a subtype of LAD with dual IgA and IgG autoimmune response to BP180.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoantibodies / blood*
  • Autoantigens / immunology*
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / immunology
  • Carrier Proteins
  • Collagen Type XVII
  • Cytoskeletal Proteins
  • Diagnosis, Differential
  • Dystonin
  • Female
  • Humans
  • Immunoglobulin A / immunology
  • Immunoglobulin G / immunology
  • Nerve Tissue Proteins
  • Non-Fibrillar Collagens
  • Pemphigoid, Bullous / immunology*

Substances

  • Autoantibodies
  • Autoantigens
  • Carrier Proteins
  • Cytoskeletal Proteins
  • DST protein, human
  • Dystonin
  • Immunoglobulin A
  • Immunoglobulin G
  • Nerve Tissue Proteins
  • Non-Fibrillar Collagens