Pemphigus Foliaceus Medication

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 15, 2012
 

Medication Summary

Numerous medications are used to treat patients with pemphigus foliaceus. They are often used in combination. However, a systematic review and meta-analysis according to the methodology of the Cochrane Collaboration showed that available evidence is inadequate at present to ascertain the optimal therapy for pemphigus foliaceus.[46]

Pemphigus is usually treated with systemic prednisone in combination with adjuvant immunosuppressants.[47] Refractory pemphigus foliaceus has been treated with the anti-CD20 monoclonal antibody rituximab.[48, 49] Intravenous immunoglobulin (IVIg) may aslo be used because it lowers serum levels of pemphigus antibodies.[50] Concurrent administration of a cytotoxic drug appears to be beneficial.

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Corticosteroid agents

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

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Prednisone (Deltasone, Orasone)

 

Synthetic adrenocortical steroid with predominantly glucocorticoid properties. Immunosuppressant for the treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

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Antibiotic agents

Class Summary

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

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Minocycline (Dynacin, Minocin)

 

Semisynthetic derivative of tetracycline. Treats infections caused by susceptible gram-negative and gram-positive organisms, in addition to infections caused by susceptible Chlamydia, Rickettsia, and Mycoplasma species. Was found to be effective in some nontuberculotic mycobacterial infections.

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Dapsone (Avlosulfon)

 

Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Used to control the dermatologic symptoms of dermatitis herpetiformis. Can be used for patients with pemphigus and may be DOC for PH and IgA PF. May be provided as monotherapy or in combination with systemic steroids and immunosuppressants.

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Antimalarial agents

Class Summary

Hydroxychloroquine has immunosuppressive effects.

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Hydroxychloroquine (Plaquenil)

 

4-Aminoquinoline derivative active against a variety of autoimmune disorders. Inhibits chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions. Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.

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Immunomodulatory agents

Class Summary

These agents have antiproliferative and immunosuppressive effects.

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Azathioprine (Imuran)

 

May be used alone or as steroid-sparing agent. Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

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Cyclophosphamide (Cytoxan, Neosar)

 

May be used as monotherapy or as a steroid-sparing agent. Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Slawomir Majewski, MD  Professor and Director, Department of Dermatology and Venereology, Warsaw School of Medicine, Poland

Disclosure: Nothing to disclose.

Sebastian S Majewski, MD  Consulting Staff, Department of Dermatology, Military Institute of Health Services, Warsaw, Poland

Disclosure: Nothing to disclose.

Specialty Editor Board

Robin Travers, MD  Assistant Professor of Medicine (Dermatology), Dartmouth University School of Medicine; Staff Dermatologist, New England Baptist Hospital; Private Practice, SkinCare Physicians

Robin Travers, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Informatics Association, Massachusetts Medical Society, Medical Dermatology Society, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Julia R Nunley, MD  Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic 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, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

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Middle-aged American woman of Mexican lineage with superficial bullae characteristic of pemphigus foliaceus.
Pemphigus foliaceus. Middle-aged American woman of Mexican lineage with superficial bullae formation.
A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.
A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.
Histologic view shows the typical pattern of a detached stratum corneum without bullae formation. Pigmentary incontinence is prominent in the dermis, reflecting the patient's 9-year history of recurrent superficial bullae.
 
 
 
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