Bullous Systemic Lupus Erythematosus (BSLE) Treatment & Management

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

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. [3, 33] 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. Dapsone has the potential adverse effects of hemolytic anemia and agranulocytosis, especially in patients with a history of anemia and leukopenia.

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. [25]

Methotrexate (MTX), azathioprine, mycophenolate mofetil, and rituximab represent additional therapeutic options. [34, 35, 36]

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. [35, 37]

Extensive eruptions of toxic epidermal necrolysis–like lupus erythematosus require prompt institution of therapy with intravenous immunoglobulin (IVIG) and/or systemic corticosteroids. [38] IVIG is a viable treatment option when dapsone is contraindicated. [39] However, further studies are needed to determine the true efficacy and safety of IVIG in the treatment of bullous systemic lupus erythematosus. 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.

Less fulminant manifestations of erythema multiforme–like lupus erythematosus can be treated with antimalarials (including hydroxychloroquine), 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.)

Lenalidomide, a thalidomide analogue, has been shown to be effective for a spectrum of cutaneous manifestations of systemic lupus erythematosus in adults. A small study from 2016 showed resolution of cutaneous manifestations, including bullous lesions, of pediatric systemic lupus erythematosus. [40] However, more study is needed to clarify the role of lenalidomide in treating the cutaneous manifestations of systemic lupus erythematosus in both children and adults.

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