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Bullous Systemic Lupus Erythematosus (BSLE) Medication

  • Author: Hillary D Johnson-Jahangir, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Dec 15, 2015
 

Medication Summary

Bullous systemic lupus erythematosus generally responds well to medical therapy. Dapsone represents the mainstay of treatment, typically leading to rapid clearance of lesions.

As in drug-induced toxic epidermal necrolysis, intravenous immunoglobulin represents an important therapeutic option for the Fas-mediated massive epidermal necrosis of fulminant toxic epidermal necrolysis–like lupus erythematosus.

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Antimycobacterial Agents

Class Summary

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

Dapsone

 

The general mechanism of action of dapsone is similar to that of sulfonamides, in which competitive antagonism of para-aminobenzoic acid (PABA) prevents the formation of folic acid, inhibiting bacterial growth.

The anti-inflammatory mechanism of dapsone is believed to result from suppression of neutrophils by the inhibition of neutrophil myeloperoxidase and inflammation-inducing oxygen intermediates. Dapsone has been shown to inhibit some forms of neutrophil chemotaxis, suppress leukocyte integrin function, and decrease the attachment of neutrophils to endothelial cell junctions.

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Corticosteroids

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.

Prednisone

 

Prednisone decreases inflammation; in particular, it suppresses neutrophil presence and activity at sites of inflammation by inhibition of endothelial cell adhesion molecule expression and chemoattractant production. Prednisone also inhibits antigen presentation, T lymphocyte activity, and (at higher doses) antibody production.

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Immunosuppressive Agents

Class Summary

These drugs have anti-inflammatory effects.

Methotrexate (Trexall, Rheumatrex)

 

Methotrexate is an antimetabolite that inhibits dihydrofolate reductase, thereby hindering deoxyribonucleic acid (DNA) and ribonucleic acid (RNA) synthesis in lymphocytes and other immune cells. Anti-inflammatory effects also result from the inhibition of 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR) transformylase (increasing tissue concentrations of the anti-inflammatory mediator adenosine) and methionine synthetase (reducing production of the proinflammatory mediator S-adenyl methionine).

Azathioprine (Imuran, Azasan)

 

Azathioprine antagonizes purine metabolism and inhibits the synthesis of DNA, ribonucleic acid (RNA), and proteins. It may decrease the proliferation of immune cells, in that way lowering autoimmune activity.

Mycophenolate mofetil (CellCept) or mycophenolic acid (Myfortic)

 

Mycophenolate mofetil has been shown to useful in the treatment of autoimmune diseases.

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Blood Products

Class Summary

These are used to improve the clinical and immunologic aspects of the disease. They may decrease autoantibody production and increase solubilization and removal of immune complexes.

Immune globulin intravenous (Gamunex, Octagam, Gammaplex , Gammagard)

 

This provides an antibody-mediated blockade of Fas-Fas ligand interactions involved in the epidermal necrosis of toxic epidermal necrolysis–like cutaneous lupus erythematosus.

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Antineoplastics, Anti-CD20 Monoclonal Antibodies

Rituximab (Rituxan)

 

Rituximab is an anti­CD20 monoclonal antibody that depletes the number of mature B ­cells.

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Contributor Information and Disclosures
Author

Hillary D Johnson-Jahangir, MD, PhD Clinical Assistant Professor, Department of Dermatology, University of Iowa, Roy J and Lucille A Carver College of Medicine

Hillary D Johnson-Jahangir, MD, PhD is a member of the following medical societies: American College of Mohs Surgery, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Iowa Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Alice L Ye Medical Student, University of Iowa, Carver College of Medicine

Disclosure: Nothing to disclose.

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

Julie V Schaffer, MD Assistant Professor of Dermatology and Pediatrics, Director of Dermatology Resident Education, Director of Pediatric Dermatology Unit, New York University School of Medicine

Disclosure: Nothing to disclose.

Ivan D Camacho, MD Dermatologist, Private Practice; Voluntary Assistant Professor of Dermatology, Department of Dermatology and Cutaneous Surgery, University of Miami, Leonard M Miller School of Medicine

Ivan D Camacho, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Florida Medical Association, International Society of Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Acknowledgements

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; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Michael Girardi, MD Associate Professor and Residency Director, Department of Dermatology, Yale University School of Medicine

Michael Girardi, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

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.

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Tense vesiculobullous lesions on the neck of a patient with bullous systemic lupus erythematosus.
 
 
 
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