Pemphigus, IgA Workup

  • Author: Lawrence S Chan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 9, 2010
 

Laboratory Studies

  • Skin biopsy is performed in IgA pemphigus (see Procedures).
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Other Tests

Perform other tests, if available, to document the diagnosis of IgA pemphigus, including immunoblotting, enzyme-linked immunosorbent assay (ELISA), and special immunofluorescence studies.

  • Immunoblotting in IgA pemphigus: Immunoblotting documents the specific skin antigen recognized by the patient's IgA autoantibodies. Immunoblotting has documented IgA autoantibodies (from 1 patient with IEN-type IgA pemphigus) that target desmosomal component desmoglein 3, which is located at the lower part of epidermis. However, other investigators were unable to detect IgA immunoreactivity to this protein in patient sera; therefore, the significance of the antigen remains unknown.
  • Enzyme-linked immunosorbent assay in IgA pemphigus: In theory, ELISA should be a good methodology for documenting the specific desmosomal antigen(s) recognized by patient IgA autoantibodies, since it uses native protein and increases detection sensitivity. In 2001, ELISA detected IgA autoantibodies recognizing desmoglein 3 and desmoglein 1 in a small percent of patients with IgA pemphigus.[22]
  • Special immunofluorescence tests in IgA pemphigus: Special immunofluorescence tests using cultured cells that express recombinant desmosomal component (desmocollin 1) document the presence of circulating IgA autoantibodies that recognize desmosomal component. Special immunofluorescence tests have documented IgA autoantibodies from patients with SPD-type IgA pemphigus. The tests detected desmosomal component desmocollin 1; however, this test is not generally available.
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Procedures

Skin biopsy is performed and specimens analyzed to establish the diagnosis using histopathology and direct and indirect immunofluorescence.

  • Histopathology is performed on specimens of blistered skin. Histopathology demonstrates an intraepidermal blister, which may be located subcorneally (SPD-type IgA pemphigus), suprabasally, or at mid epidermis (IEN-type IgA pemphigus). Histopathology demonstrates acantholysis, but it is not characteristic as is acantholysis observed in classic pemphigus. The intraepidermal neutrophil infiltration tends to form neutrophilic microabscess, which is the hallmark of IgA pemphigus (see the image below). Histopathologic examination of a blister lesion obHistopathologic examination of a blister lesion obtained from a patient with immunoglobulin A pemphigus shows a suprabasal blistering process, acantholysis, and an inflammatory cell infiltrate containing numerous neutrophils (hematoxylin and eosin, original magnification X50).
  • Direct immunofluorescence is performed on perilesional skin sections. Direct immunofluorescence documents the immune-mediated disease process. Direct immunofluorescence predominantly detects IgA and sometimes, to a lesser extent, IgG and complement component C3 deposited at the cell surfaces of the epidermis.
  • Indirect immunofluorescence is performed using patient serum on monkey esophagus or other epithelial substrates. Indirect immunofluorescence documents the presence of IgA circulating autoantibodies in patient serum that recognize skin epidermal cell surface components (see the image below). Indirect immunofluorescence usually detects IgA circulating autoantibodies in serum that bind to epithelial cell surfaces in approximately 50% of patients. Titers of IgA pemphigus autoantibodies are much lower than the titers of IgG autoantibodies observed in IgG-mediated pemphigus. Indirect immunofluorescence microscopy performed oIndirect immunofluorescence microscopy performed on monkey esophagus substrate (with serum from a patient with immunoglobulin A pemphigus) detects the immunoglobulin A1 subclass of circulating autoantibodies that label the epithelial cell surfaces of the substrate.
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Contributor Information and Disclosures
Author

Lawrence S Chan, MD  Dr Orville J Stone Professor of Dermatology, Head, Department of Dermatology, University of Illinois College of Medicine

Lawrence S Chan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Dermatological Association, American Medical Association, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois State Medical Society, Microcirculatory Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Takeji Nishikawa, MD  Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Histopathologic examination of a blister lesion obtained from a patient with immunoglobulin A pemphigus shows a suprabasal blistering process, acantholysis, and an inflammatory cell infiltrate containing numerous neutrophils (hematoxylin and eosin, original magnification X50).
Indirect immunofluorescence microscopy performed on monkey esophagus substrate (with serum from a patient with immunoglobulin A pemphigus) detects the immunoglobulin A1 subclass of circulating autoantibodies that label the epithelial cell surfaces of the substrate.
 
 
 
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