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Nonsecretory IgA1 autoantibodies targeting desmosomal component desmoglein 3 in intraepidermal neutrophilic IgA dermatosis.

Abstract
Intraepidermal neutrophilic IgA dermatosis, a rare skin disease entity manifested with blisters and pustules clinically and lower epidermal blister, acantholysis, and neutrophilic infiltration pathologically, was first reported in 1985. Although the disease is characterized by IgA autoantibodies targeting the epithelial cell surface component, the target antigen has not been determined. We investigated a patient with this disease by histopathology, direct and indirect immunofluorescence, immunoblotting, and immunoadsorption studies. The pustular lesion was characterized by blister at the lower epidermis, acantholysis, and neutrophilic infiltration. Nonsecretory IgA1 subclass autoantibodies targeting the lower epithelial cell surfaces were detected in the patient's skin and serum. The patient's IgA autoantibodies labeled a recombinant desmosomal protein desmoglein 3 on immunoblotting and the immunolabeling of epithelial cell surfaces was eliminated by preadsorption with desmoglein 3. Thus, desmoglein 3 is identified as a target antigen in intraepidermal neutrophilic IgA dermatosis. The ability of IgA1 autoantibodies to bind neutrophils may be responsible for the prominent neutrophilic infiltration observed histopathologically and for the pustular lesions observed clinically.
AuthorsJ Wang, J Kwon, X Ding, J A Fairley, D T Woodley, L S Chan
JournalThe American journal of pathology (Am J Pathol) Vol. 150 Issue 6 Pg. 1901-7 (Jun 1997) ISSN: 0002-9440 [Print] United States
PMID9176383 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S.)
Chemical References
  • Autoantibodies
  • Cadherins
  • DSG3 protein, human
  • Desmoglein 3
  • Epitopes
  • Immunoglobulin A
  • Recombinant Proteins
Topics
  • Aged
  • Autoantibodies (immunology)
  • Cadherins (genetics, immunology)
  • Cloning, Molecular
  • Desmoglein 3
  • Epitopes
  • Fluorescent Antibody Technique, Direct
  • Humans
  • Immunoblotting
  • Immunoglobulin A (biosynthesis)
  • Immunosorbent Techniques
  • Male
  • Pemphigus (immunology, pathology)
  • Recombinant Proteins (metabolism)
  • Skin (immunology)

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