Paraneoplastic Pemphigus Medication

Updated: Feb 07, 2019
  • Author: Lynne J Goldberg, MD; Chief Editor: Dirk M Elston, MD  more...
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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.



Class Summary

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

Prednisone (Deltasone, Orasone, Sterapred)

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

Azathioprine (Imuran)

Azathioprine is often used in conjunction with prednisone for dermatologic purposes as a steroid-sparing agent. It is a purine analog with cytotoxic properties. The prodrug is metabolized to 6-mercaptopurine. It inhibits RNA and DNA synthesis.

Cyclosporine (Sandimmune, Neoral)

Cyclosporine has been demonstrated to be helpful in a variety of skin disorders. It is a potent immunosuppressive agent most often used in organ transplantation. Cyclosporine diminishes the production of autoantibodies and decreases resultant blistering and erosions. It acts by inhibiting T lymphocytes and lymphokine production.

Cyclophosphamide (Cytoxan, Neosar)

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


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.

Immune globulins intravenous (Gammagard, Gamimune, Sandoglobulin)

Immune globulins intravenous is prepared from the pooled plasma of 10,000-20,000 donors. It 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. It has been used for dermatomyositis, pemphigus foliaceus, pemphigus vulgaris, epidermolysis bullosa acquisita, bullous pemphigoid, and herpes gestationis. A high dose is needed for treatment of inflammatory and autoimmune disorders in comparison to replacement therapy for patients with deficiency.