Bullous systemic lupus erythematosus associated with increased activity of lupus nephritis: a case report and review of the literatur

Bullous systemic lupus erythematosus (BSLE) is a rare subepidermal blistering disease with distinctive histological and immunological patterns. The disease affects mainly young females. The typical presentation is vesicobullous eruption with an acute onset and predilection for the face, trunk, upper extremities and vermillion border [1].
Bullous systemic lupus erythematosus can be the initial presentation of SLE or it may be associated with SLE flare [2, 3]. The association between BSLE and increased activity of lupus nephritis has been particularly underscored by several authors [4–9]. Herein, we present a patient diagnosed with lupus nephritis several years earlier, in whom the development of BSLE was associated with the recurrence of renal disease.
A 22-year-old woman was admitted to the Department of Dermatology in January 2016 for evaluation of disseminated asymptomatic erythematous lesions with numerous small vesicles, which had developed one month prior to hospitalization. The patient had been diagnosed with lupus nephritis in 2006 and since then treated with systemic corticosteroids, eventually tapered off to a maintenance dose of 4 mg/day of methylprednisolone. No skin lesions had been present in the past. The routine blood cell count and urine analysis, performed one month prior to the development of skin lesions, had been within normal limits and there had been no symptoms of SLE activity. On admission, physical examination revealed disseminated erythematous lesions with numerous tense fluid-filled vesicles, erosions and crusts located on the face, neck, upper part of the trunk and upper extremities (Figures 1 A, B). Nikolsky’s sign was negative. No malar rash or discoid rash were present. Laboratory investigations revealed a decreased concentration of hemoglobin (10.6 g/dl) and red blood cells count (3.61 × 106/µl), hematuria with proteinuria (3.3 g/day), hypoproteinemia (4.8 g/dl; normal range: 6.4–8.3 g/dl) with hypoalbuminemia (2.7 g/dl, normal range: 3.5–5.2 g/dl), decreased levels of C3 (0.252 g/l; normal range: 0.9–1.8 g/l) and C4 (0.0358 g/l; normal range: 0.1–0,4 g/l) and presence of antinuclear antibodies (ANA), including anti-double-stranded DNA (anti-dsDNA) antibodies. Direct immunofluorescence showed markedly positive granular deposits of IgM, C1q and C3 with discrete IgG and IgA at the dermoepidermal junction. No circulating antibodies were detected on indirect immunofluorescence. Subepidermal blister and...


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