Epidermolysis Bullosa Acquisita Medication

  • Author: Lawrence S Chan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Nov 15, 2011
 

Medication Summary

In epidermolysis bullosa acquisita(EBA), as in other autoimmune diseases, treatment is directed at decreasing the development of new blisters, promoting healing, and preventing scarring and the sequelae of scarring.

For an autoimmune disease, such as epidermolysis bullosa acquisita, the logical approach for treatment is to modify or reduce the autoimmune responses, and to decrease the production of autoantibodies.

A target-specific immunomodulatory treatment that blocks the autoimmune response would be the treatment of choice and remains the holy grail of investigators in the field.

To date, only non–target-specific immunosuppressive and anti-inflammatory agents[28] are available.

Mycophenolate mofetil, a drug with a similar mechanism of action to azathioprine, is being used with increasing frequency to treat autoimmune diseases. It may prove to be a viable alternative to azathioprine, although more data are needed.

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Anti-inflammatory agents

Class Summary

In the inflammatory form of EBA, there may be an inflammatory cell infiltration near the basement membrane zone. Theoretically, anti-inflammatory agents would block the inflammatory process and improve the disease.

In the author's experience, systemic steroids have not proved to be very beneficial in treating EBA.

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Prednisone (Deltasone)

 

Used as sole agent or in conjunction with other medications (eg, immunosuppressives) to treat EBA.

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production.

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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 in conjunction with other anti-inflammatory medications and immunosuppressives.

In children, physicians should consult the patient's pediatrician before prescribing this medication.

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Immunosuppressive agents

Class Summary

EBA is an autoimmune disease, targeting the skin basement membrane component type VII collagen. Immunosuppressives are used in patients with severe disease. Immunomodulatory medications or therapies, if used properly, may be able to correct this alteration.

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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.

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Photophoresis agents

Class Summary

Is a therapeutic method that uses UV-sensitizing medication (psoralen) and extracorporeal ultraviolet A irradiation of the sensitized WBCs. The photoinactivated cells are reinfused into the patient.

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Methoxsalen (8-MOP, Oxsoralen)

 

Inhibits mitosis by binding covalently to pyrimidine bases in DNA when photoactivated by UV-A.

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Biological response modulators

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Rituximab (Rituxan)

 

Rituximab is a monoclonal antibody (mostly human origin, partly mouse protein) that is specifically targeting an immune cell surface marker termed CD20, which is primarily expressed by B lymphocytes (B cells). The antibody is an IgG class immunoglobulin with kappa light chain.

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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.

Coauthor(s)

David Woodley, MD  Co-Chair, Professor, Department of Medicine, Division of Dermatology, University of Southern California

David Woodley, MD is a member of the following medical societies: American Academy of Dermatology, American Association for the Advancement of Science, American College of Emergency Physicians, American College of Physicians, American Federation for Medical Research, American Society for Clinical Investigation, New York Academy of Medicine, Society for Investigative Dermatology, and Southern Medical Association

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

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

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.

References

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Direct immunofluorescence performed on perilesional skin biopsy specimen from a patient with epidermolysis bullosa acquisita detects a linear band of immunoglobulin G deposit along the dermoepidermal junction.
Indirect immunofluorescence performed on salt-split normal human skin substrate using serum from a patient affected with epidermolysis bullosa acquisita detects immunoglobulin G class circulating autoantibodies that bind to the dermal (base) side of the basement membrane.
 
 
 
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