Bullous Systemic Lupus Erythematosus (BSLE) Treatment & Management
- Author: Hillary D Johnson-Jahangir, MD, PhD; Chief Editor: Dirk M Elston, MD more...
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.
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.
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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