Pemphigus Herpetiformis Workup

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

Laboratory Studies

  • Diagnosis of pemphigus herpetiformis: To establish a diagnosis of pemphigus herpetiformis, perform the following tests: histopathology on newly blistered skin, direct immunofluorescence (DIF) on periblistered skin, and indirect immunofluorescence (IIF) of the patient's sera on monkey esophagus substrate.
  • Histopathology of pemphigus herpetiformis: Histopathology documents the acantholytic process and inflammatory cell infiltration (eosinophils, neutrophils, or both) that characterize pemphigus herpetiformis. Note the image below Histopathologic examination of a blister lesion obHistopathologic examination of a blister lesion obtained from a patient with pemphigus herpetiformis shows a subcorneal blistering process, acantholysis, and neutrophilic infiltrate.
  • Direct immunofluorescence in pemphigus herpetiformis (see image below): DIF documents the immunologic nature of pemphigus herpetiformis. DIF usually detects the presence of IgG with or without C3 deposition around cell surfaces of keratinocytes in the patient's skin. The optimal location for the skin biopsy used in this study is adjacent to a blister or an inflammatory papule. Direct immunofluorescence microscopy performed on Direct immunofluorescence microscopy performed on a skin biopsy specimen obtained from a patient with pemphigus herpetiformis detects immunoglobulin G deposits on the epithelial cell surfaces but sparing the basal layers.
  • Indirect immunofluorescence in pemphigus herpetiformis: IIF confirms the presence in the patient's serum of IgG-circulating autoantibodies that bind to epidermal cell surfaces. In some patients, IgG autoantibodies predominantly bind to the upper part of the epidermis; in other patients, IgG autoantibodies bind to the entire epidermis. The titer of autoantibodies often parallels the clinical level of activity of pemphigus herpetiformis and often is helpful in following the patient's disease activity after the diagnosis has been established.
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Imaging Studies

  • Patients with pemphigus herpetiformis who have associated pulmonary symptoms should undergo routine chest radiography. CT scanning may also be needed.
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Other Tests

  • Immunoblotting in pemphigus herpetiformis[15] : Immunoblotting documents specific skin desmosomal components, in a denatured form, that are recognized by the patient's IgG autoantibodies. Most patients with pemphigus herpetiformis have IgG autoantibodies that recognize desmoglein 1, a desmosomal protein predominantly located in the upper epidermis. In a minority of patients with pemphigus herpetiformis, IgG autoantibodies were detected to desmoglein 3, a desmosomal protein predominantly located in the lower epidermis.
  • Enzyme-linked immunosorbent assay in pemphigus herpetiformis[16] : Similar to immunoblotting, enzyme-linked immunosorbent assay (ELISA) detects the specific protein component (usually in recombinant nondenatured form) recognized by the patient's IgG autoantibodies. ELISA usually is more sensitive than immunoblotting in detecting the protein recognized by autoantibodies.
  • Immunoprecipitation in pemphigus herpetiformis: Immunoprecipitation has the same function as immunoblotting but is more difficult to perform. Immunoprecipitation detects the native form of the target antigen rather than the denatured form of the antigen.
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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

Smeena Khan, MD  Private Practice, Adult and Pediatric Dermatology Associates

Smeena Khan, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

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 pemphigus herpetiformis shows a subcorneal blistering process, acantholysis, and neutrophilic infiltrate.
Direct immunofluorescence microscopy performed on a skin biopsy specimen obtained from a patient with pemphigus herpetiformis detects immunoglobulin G deposits on the epithelial cell surfaces but sparing the basal layers.
 
 
 
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