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Bullous Systemic Lupus Erythematosus (BSLE) Treatment & Management

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

Approach Considerations

Bullous systemic lupus erythematosus generally responds well to medical therapy, and treatment with dapsone is particularly effective. Although type 1 bullous systemic lupus erythematosus and epidermolysis bullosa acquisita are characterized by antibodies targeting type VII collagen, epidermolysis bullosa acquisita differs considerably in its marked resistance to therapy.

A dermatologist may be consulted for the evaluation and management of bullous systemic lupus erythematosus, toxic epidermal necrolysis, erythema multiforme–like lupus erythematosus, or another cutaneous manifestation of lupus erythematosus.

An internist/rheumatologist may be consulted for the evaluation and management of extracutaneous (eg, renal, cardiac, pulmonary) manifestations of systemic lupus erythematosus.

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Pharmacologic Therapy

Dapsone is the initial treatment of choice for bullous systemic lupus erythematosus. The response is usually dramatic, with cessation of new blister formation within 1-2 days and rapid healing of existing lesions. Low doses (25-50 mg/d) are often effective, although a higher dosage is sometimes required. Rapid recurrences may occur upon withdrawal of dapsone, with prompt remission after reinstitution of therapy. However, discontinuance of dapsone therapy is usually possible within a year.

Prednisone may be effective in patients who cannot tolerate dapsone (eg, those with glucose-6-phosphate dehydrogenase [G-6-PD] deficiency), have a poor response to dapsone, or require treatment of concurrent systemic manifestations of systemic lupus erythematosus. Combination therapy with prednisone and dapsone can also be beneficial.  For patients with parallel exacerbations such as lupus nephritis, additional therapeutics such as corticosteroids and other immunosuppressive agents should be considered.[20]

Methotrexate (MTX), azathioprine, mycophenolate mofetil, and rituximab represent additional therapeutic options.[28, 29, 30]

Rituximab is an anti-CD20 monoclonal antibody hypothesized to reduce the number of antitype VII collagen antibodies by depletion of mature B cells. It may useful for select patients who do not respond to dapsone or other immunosuppressive agents.[29, 31]

Extensive eruptions of toxic epidermal necrolysis–like lupus erythematosus require prompt institution of therapy with intravenous immunoglobulin and/or systemic corticosteroids. Less fulminant manifestations of erythema multiforme–like lupus erythematosus can be treated with antimalarials, corticosteroids (topical or systemic, depending on the severity and presence of systemic disease), and other agents in the therapeutic armamentarium for lupus erythematosus. (See also Subacute Cutaneous Lupus Erythematosus.)

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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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