Bullous systemic lupus erythematosus (BSLE) is a rare autoantibody-mediated bullous dermatosis, commonly associated with autoimmunity to type VII collagen [1]. It occurs in a subset of individuals with systemic lupus erythematosus, at an estimated incidence of 0.26 cases per million per year [2]. Due to the low incidence of BSLE, published data regarding treatment options has been limited to case reports and small case series. These have reported dapsone, systemic corticosteroids, cyclophosphamide, azathioprine, rituximab, and methotrexate as potential therapeutic options. Typically, these patients are already on systemic agents to manage their SLE, and hence, when the blistering is under control, often they have to maintain some treatment to prevent the SLE from relapsing.

62.2 Dapsone

Dapsone is a sulfone derivative with antimicrobial and anti-inflammatory properties that is often considered the drug of choice for BSLE. Currently, there are 28 cases reported in the literature of dapsone being used either alone or in conjunction with other immunosuppressive agents, with 26 of 28 patients (93 %) experiencing an improvement on dapsone therapy [3–23]. Typically, there is complete remission with a dramatic improvement occurring within days of commencing 50–100 mg/day of oral dapsone, although two cases did report a benefit only after 2 weeks of treatment [11, 22]. Two patients (7 %) did not improve with therapy, with the lesions in one patient reportedly worsening with dapsone therapy [6, 9].

Doses as low as 25 mg/day have been reported to be effective [17, 19], although up to 150 mg/day may be required before an improvement is seen [22, 23]. Complete healing may occur without scarring, milia, or pigmentary changes [18, 19], but residual post-inflammatory hyperpigmentation may often occur [11, 23]. Maintenance doses of between 50 and 200 mg/day have been reported to be effective in preventing relapses of bullous lesions [16, 19, 22, 23]. Relapse can sometimes occur when dapsone is ceased or the dose is tapered [8, 22] and may occur up to 5 months after stopping therapy [17]. However, sustained remission has also been reported following dapsone cessation [3, 14, 19].

Among the 26 patients showing an improvement with dapsone, 14 patients (54 %) had failed to respond to high-dose systemic corticosteroid therapy prior to commencement of dapsone [3, 4, 8, 14, 16–19, 21–23]. All 14 patients subsequently improved when dapsone was initiated as an adjunct. In all of the remaining 12 patients who showed an improvement, dapsone monotherapy appears to have been successfully used as a first-line therapy [5, 7, 8, 10–13, 15].

62.3 Corticosteroid Monotherapy

Systemic glucocorticoids such as prednisolone or methylprednisolone have largely been ineffectual in the treatment of BSLE. The 14 cases mentioned above failed to improve with corticosteroid therapy, and only three additional cases (18 %) have reported a successful regression of bullous lesions with corticosteroid monotherapy [24–26]. These cases reported slow improvement only after prolonged treatment with prednisone lasting several weeks, at doses varying between 45 mg/day and 0.75 mg/kg/day, which were then progressively tapered [24–26].

Interestingly, prednisone hypersensitivity has been reported to occur rarely in the setting of systemic lupus erythematosus, with new bullous lesions appearing in a 14-year-old girl due to erythema multiforme associated with prednisone hypersensitivity, rather than BSLE [27].

62.4 Corticosteroids with Cyclophosphamide

Three case reports have examined combination treatment with cyclophosphamide and corticosteroids. One patient treated with methylprednisolone (250–500 mg/day orally) and pulses of cyclophosphamide (600–800 mg intravenously) demonstrated an improvement in his bullous lesions with no recurrence of skin disease [28]. However, the other two cases failed to respond to pulsed cyclophosphamide intravenously and high-dose systemic corticosteroids, and both improved only after the addition of dapsone at 50–75 mg/day [8, 17]. In general, due to the risk of adverse effects, cyclophosphamide is considered to be a treatment of last resort for BSLE.

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