Bullous Systemic Lupus Erythematosus (BSLE) Medication

Updated: Mar 13, 2018
  • Author: Sarah Sweeney Pinney, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Medication

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. Dapsone has the potential adverse effects of hemolytic anemia and agranulocytosis, especially in patients with a history of anemia and leukopenia.

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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