Sections

Medication

Medication Summary

The aim of treatment is to reduce the inflammatory response and autoantibody production. While target-specific therapy is not available, non–target-specific treatments currently are used. The most commonly used medications are corticosteroids.

The introduction of corticosteroids has reduced mortality greatly, but significant morbidity remains. Immunosuppressants should be considered early in the course of disease, as steroid-sparing agents. Mycophenolate mofetil and azathioprine are the usual agents considered as initial choices.^{[70, 71]}Some suggest measuring levels of thiopurine methyltransferase (TPMT), a key enzyme in azathioprine metabolism, before starting patients on azathioprine.^{[72, 73, 74]}Only one placebo-controlled blinded study of mycophenolate has demonstrated more rapid improvement in the short run but no significant steroid-sparing effects in the long term.^[75]A retrospective chart review has suggested a therapeutic ladder for patients with pemphigus vulgaris, but these authors' approach has not been validated.^[76]Rituximab and intravenous immunoglobulin have also proven useful alone or in combination, and some authorities are now using rituximab as first-line therapy for severe disease.^[77]Rituximab was approved by the FDA for the treatment of pemphigus vulgaris in June 2018.^[78]

A small case control trial showed improved laboratory and clinical outcomes in patients with pemphigus vulgaris treated with a combination of cytotoxic agents plus intravenous immunoglobulin (IVIG)compared with those treated with just IVIG.^[79]

Cyclophosphamide is used for refractory disease.^[80]The role of biologic agents is being investigated. Each of these agents should be prescribed and monitored by physicians familiar with them. Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring.

Class Summary

Monoclonal antibodies target the CD20 antigen expressed on the surface of pre-B and mature B-lymphocytes. B cells may be acting at multiple sites in the autoimmune/inflammatory process, including T-cell activation and/or proinflammatory cytokine production.

Rituximab (Rituxan)

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Rituximab is an anti-CD20 monoclonal antibody indicated for adults with pemphigus vulgaris in combination with short-term corticosteroid therapy.

Class Summary

Anti-inflammatory agents inhibit the inflammatory process by inhibiting specific cytokine production.

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

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Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. It stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. For treating pemphigus vulgaris, administer it orally and use alone or in combination with topical or intralesional steroids or in conjunction with other immunosuppressives.

In pediatric patients, disease management with this medication in consultation with the patient's pediatrician is advised.

Class Summary

Immunosuppressive agents are useful adjuvants in patients with pemphigus vulgaris with generalized disease unresponsive to steroids and/or other anti-inflammatory agents or in patients unable to tolerate prednisone. Some suggest measuring levels of thiopurine methyltransferase (TPMT), a key enzyme in azathioprine metabolism, before starting patients on azathioprine.

Azathioprine (Imuran)

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Azathioprine antagonizes purine metabolism and inhibits the synthesis of DNA, RNA, and proteins. It may decrease the proliferation of immune cells, which results in lower autoimmune activity. In conjunction with prednisone, it is more effective than prednisone alone. OFf-label use of azathioprine may be an effective monotherapy in mild cases, although therapeutic effects are delayed 3-5 weeks. Consider withdrawal if no improvement is seen within 3 months.

Questions

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Media Gallery

Early, small blister filled with clear fluid arises on healthy skin.
Flaccid blister filled with clear fluid arises on healthy skin.
An erosion.
Erosions and healing areas on the back.
Healing areas on the chest and abdomen.
Direct immunofluorescence showing intercellular immunoglobulin G throughout the epidermis of a patient with pemphigus vulgaris.

of 6

Author

Bassam Zeina, MD, PhD Consulting Staff, Department of Dermatology, Milton Keynes Hospital, UK

Bassam Zeina, MD, PhD is a member of the following medical societies: British Medical Association, Royal Society of Medicine, British Association of Dermatologists

Disclosure: Nothing to disclose.

Coauthor(s)

Nicole Sakka, MBBS Foundation Year 2, Royal Liverpool and Broadgreen University Hospital, Liverpool, UK

Disclosure: Nothing to disclose.

Sohail Mansoor, MBBS, MSc Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK

Sohail Mansoor, MBBS, MSc is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, Royal College of Physicians, American Academy of Anti-Aging Medicine, Royal College of Physicians and Surgeons of Glasgow

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

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, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Abby S Van Voorhees, MD Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, Women's Dermatologic Society, National Psoriasis Foundation, American Medical Association, Phi Beta Kappa, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbott for consulting; Partner received salary from Merck for management position; Received honoraria from Abbott for speaking and teaching; Received honoraria from Amgen for review panel membership; Received honoraria from Centocor for consulting; Received honoraria from Leo for consulting; Received none from Merck for other.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Mohsin Ali, MBBS, FRCP, MRCP, to the development and writing of this article.

Pemphigus Vulgaris Medication

Medication Summary

Anti-CD20 Monoclonal Antibodies

Class Summary

Rituximab (Rituxan)

Rituximab (Rituxan)

Anti-inflammatory agents

Class Summary

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Immunosuppressive agents

Class Summary

Azathioprine (Imuran)

Azathioprine (Imuran)

Pemphigus Vulgaris Medication

Medication Summary

Anti-CD20 Monoclonal Antibodies

Class Summary

Rituximab (Rituxan)

Rituximab (Rituxan)

Anti-inflammatory agents

Class Summary

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Immunosuppressive agents

Class Summary

Azathioprine (Imuran)

Azathioprine (Imuran)

References

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