Paraneoplastic Pemphigus Medication

  • Author: Lynne J Goldberg, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 13, 2012
 

Medication Summary

The medications used to treat paraneoplastic pemphigus are potent immunosuppressive agents with numerous adverse effects. All patients taking these medications require periodic monitoring of laboratory values.

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Immunosuppressives

Class Summary

These agents diminish the production of autoantibodies and decrease resultant blistering and erosions.

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Prednisone (Deltasone, Orasone, Sterapred)

 

Affects all organ systems. On a cellular level, affects cell activation, replication, differentiation, and mobility. Net result is inhibition of immediate and delayed hypersensitivity. Suppression of antibody production requires higher doses than suppression of monocyte function.

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Azathioprine (Imuran)

 

Often used in conjunction with prednisone for dermatologic purposes as a steroid-sparing agent. Purine analog with cytotoxic properties. Prodrug metabolized to 6-mercaptopurine. Inhibits RNA and DNA synthesis.

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Cyclosporine (Sandimmune, Neoral)

 

Demonstrated to be helpful in a variety of skin disorders. Potent immunosuppressive agent most often used in organ transplantation. Diminishes production of autoantibodies and decreases resultant blistering and erosions. Acts by inhibiting T lymphocytes and lymphokine production.

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Cyclophosphamide (Cytoxan, Neosar)

 

Recent reports suggest that cyclophosphamide in combination with systemic steroids is a useful regimen for paraneoplastic pemphigus. Has been used to ablate bone marrow, followed by stem cell rescue (peripheral blood stem cell transplantation). Chemically related to nitrogen mustards. As an alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. Many PO/IV regimens exist, depending on disease being treated and status of patient.

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Immunomodulatory agents

Class Summary

IVIG is being increasingly used in high doses to treat many dermatologic inflammatory and autoimmune diseases, including autoimmune bullous disorders and dermatomyositis. However, few controlled trials exist, and it is costly and time consuming to administer. IVIG has been used as monotherapy and as adjunctive therapy.

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Immune globulins intravenous (Gammagard, Gamimune, Sandoglobulin)

 

Prepared from pooled plasma of 10,000-20,000 donors. Has many mechanisms of action, which are mediated by the Fc portion of IgG or the antigen-binding and variable regions of the F (ab') 2 portion. Has been used for dermatomyositis, pemphigus foliaceus, pemphigus vulgaris, epidermolysis bullosa acquisita, bullous pemphigoid, and herpes gestationis. High dose is needed for treatment of inflammatory and autoimmune disorders in comparison to replacement therapy for patients with deficiency.

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Contributor Information and Disclosures
Author

Lynne J Goldberg, MD  Professor, Departments of Dermatology and Pathology, Boston University School of Medicine

Lynne J Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, International Society of Dermatopathology, International Society of Dermatopathology, Massachusetts Academy of Dermatology, New England Dermatological Society, North American Hair Research Society, Phi Beta Kappa, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Nauman Nisar  MD, Dermatopathologist, Mercy Medical Center, Sioux City, IA

Nauman Nisar is a member of the following medical societies: College of American Pathologists

Disclosure: Nothing to disclose.

Specialty Editor Board

Ponciano D Cruz Jr, MD  Vice-Chair, JB Shelmire Professor, Department of Dermatology, University of Texas Southwestern Medical Center

Ponciano D Cruz Jr, MD is a member of the following medical societies: Texas Medical Association

Disclosure: RCTS Consulting fee Independent contractor; Mary Kay Cosmetics Honoraria Consulting; Galderma Grant/research funds Principal Investigator

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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 P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Celgene Honoraria Safety Monitoring Committee

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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