eMedicine Specialties > Dermatology > Bullous Diseases

Pemphigus Herpetiformis: Differential Diagnoses & Workup

Author: Lawrence Chan, MD, Department Head and Director of Skin Immunology Research, Professor, Departments of Dermatology and Microbiology/Immunology, University of Illinois College of Medicine
Contributor Information and Disclosures

Updated: Oct 15, 2008

Differential Diagnoses

Bullous Pemphigoid
Dermatitis Herpetiformis
Pemphigus Erythematosus
Pemphigus Foliaceus
Pemphigus Vulgaris

Other Problems to Be Considered

Linear immunoglobulin A bullous dermatosis

Workup

Laboratory Studies

  • 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: Histopathology documents the acantholytic process and inflammatory cell infiltration (eosinophils, neutrophils, or both) that characterize the disease.
  • Direct immunofluorescence
    • DIF documents the immunologic nature of the disease.
    • 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.
  • Indirect immunofluorescence
    • 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 the disease and often is helpful in following the patient's disease activity after the diagnosis has been established.

Imaging Studies

  • Patients who have associated pulmonary symptoms should undergo routine chest radiography. CT scanning may also be needed.

Other Tests

  • Immunoblotting12
    • 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 assay13
    • 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: 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.

More on Pemphigus Herpetiformis

Overview: Pemphigus Herpetiformis
Differential Diagnoses & Workup: Pemphigus Herpetiformis
Treatment & Medication: Pemphigus Herpetiformis
Follow-up: Pemphigus Herpetiformis
Multimedia: Pemphigus Herpetiformis
References
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References

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Further Reading

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Keywords

pemphigus herpetiformis, pemphigus, herpetiform pemphigus

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Contributor Information and Disclosures

Author

Lawrence Chan, MD, Department Head and Director of Skin Immunology Research, Professor, Departments of Dermatology and Microbiology/Immunology, University of Illinois College of Medicine
Lawrence Chan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois State Medical Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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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