Pemphigus Herpetiformis Clinical Presentation

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

History

  • Patients affected with pemphigus herpetiformis usually have a subacute onset of disease. Approximately half the patients experience severe pruritus.
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Physical

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Causes

  • Pemphigus herpetiformis appears to be mediated by IgG autoantibodies targeting the interepidermal cell adhesion molecules desmoglein 1 or desmoglein 3.[5, 6, 7] However, unlike typical pemphigus vulgaris, significant numbers of inflammatory cells, eosinophils, neutrophils, or mixed eosinophils and neutrophils infiltrate pemphigus herpetiformis lesional skin. The roles of inflammatory cells, particularly eosinophils, in the pathogenesis of pemphigus herpetiformis require further investigation.
  • Regarding the etiology in the neutrophil-dominant subset of pemphigus herpetiformis, in a study of 2 patients, IgG autoantibodies that recognized desmoglein 1 (a predominant upper-epidermal protein) exclusively were found to co-localize with expression of IL-8 (a strong neutrophil chemokine) and neutrophilic infiltration at the upper epidermis of the patients' skin. In the study of 2 patients, the purified IgG fraction of the patients' sera induced IL-8 secretion from normal cultured human keratinocytes. Purified IgG from one of these patients also induced the cytoplasmic expression of IL-8 in normal cultured human keratinocytes. Thus, in the neutrophil-dominant subset of pemphigus herpetiformis, it seems that IgG autoantibodies targeting desmoglein 1 were responsible for neutrophil recruitment to the epidermis as a result of inducing epidermal-cell IL-8 expression and secretion. Once at the epidermis, infiltrating neutrophils may result in blisters by releasing their proteases.
  • In the eosinophil-dominant subset of pemphigus herpetiformis, eosinophilic involvement has not been investigated yet.
  • One report of a patient with pemphigus herpetiformis has been associated with use of medication (thiopronine). A detailed mechanism is not delineated.[8]
  • One patient with pemphigus herpetiformis had a history of psoriasis and developed the pemphigus disease shortly after a course of ultraviolet light treatment, raising a possible role of ultraviolet light in the induction of pemphigus herpetiformis.[9]
  • At least 2 reported patients with pemphigus herpetiformis had coexisting psoriasis,[10] a chronic inflammatory dermatosis, raising a possible role of "epitope spreading" in the induction of pemphigus herpetiformis.[11]
  • Pemphigus herpetiformis may also demonstrate autoantibodies targeting antigen (or antigens) other than the typical desmoglein proteins (desmoglein 1 or 3).[12]
  • A patient with pemphigus herpetiformis, manifested by prominent skin involvement with no oral mucosal involvement, demonstrated autoantibodies to desmoglein 3, in contrast with what would typically be seen with this antibody.[13]
  • Pemphigus herpetiformis may also present with lesions characteristic of the inflammatory stage of heritable pretibial dystrophic epidermolysis bullosa.[14]
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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.

References

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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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