Pemphigus Herpetiformis Medication

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

Medication Summary

Because pemphigus herpetiformis is a rare variant of pemphigus, comprehensive therapeutic studies of large numbers of patients have not taken place. The following treatment options are taken from the personal experiences of physician-investigators with a small number of patients.

In general, dapsone is the drug of choice for pemphigus herpetiformis treatment. Other anti-inflammatory and immunosuppressive medications are also effective.

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Anti-inflammatories

Class Summary

Because pemphigus herpetiformis lesions are characterized by prominent infiltration of inflammatory cells, anti-inflammatory medication may inhibit the functions of these cells, therefore improving disease conditions.

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Dapsone (Avlosulfon)

 

Mechanism of action is similar to that of sulfonamides, in which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Used alone or in conjunction with other anti-inflammatory medication or immunosuppressives for pemphigus herpetiformis.

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Prednisone (Deltasone)

 

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production. Taken alone, or in conjunction with other anti-inflammatory or immunosuppressive medications, is useful for controlling pemphigus herpetiformis.

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Immunosuppressives

Class Summary

Pemphigus herpetiformis is an autoimmune disease; immunosuppressives are useful in suppressing autoimmune response. Also see the clinical guideline summary from the British Association of Dermatologists, Guidelines for prescribing azathioprine in dermatology.

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Azathioprine (Imuran)

 

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity. Usually used as supplement to anti-inflammatory medication; is useful in controlling pemphigus herpetiformis.

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Biological Response Modulator

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Rituximab (Rituxan)

 

Genetically engineered chimeric murine/human monoclonal antibody against human CD20, a molecule present in normal and malignant B lymphocytes. Described in case reports as a promising biological treatment for B-lymphocyte–mediated diseases (eg, pemphigus vulgaris).

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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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