Clinical, histological, immunological presentations and outcomes of bullous systemic lupus erythematosus: 10 New cases and a literature review of 118 cases

Risi-Pugliese, T. De; Aubart, F. Cohen; Haroche, Julien; Moguelet, Philippe; Grootenboer‐Mignot, Sabine; Mathian, Alexis; Ingen‐Housz‐Oro, Saskia; Hié, M.; Wendremaire, Noémie; Aucouturier, Françoise; Lepelletier, F.; Miyara, Makoto; Bader‐Meunier, Brigitte; Rémy, Philippe; Fabien, Nicole; Françès, Camille; Barète, Stéphane; Amoura, Zahir

doi:10.1016/j.semarthrit.2017.11.003

Cited by 70 publications

(108 citation statements)

References 73 publications

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“…The correlation between BSLE and SLE activity remains controversial. Nonetheless, concomitant lupus nephritis, hematologic, and neuropsychiatric involvement have been reported [ 1 , 3 , 5 ]. In a multicenter retrospective study on BSLE patients by de Risi-Pugliese et al, lupus nephritis was reported in 50% of the cases, mostly class III or IV, according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification.…”

Section: Discussionmentioning

confidence: 99%

“…Hematological involvement was found in 45% of cases at the time of BSLE. Moreover, the median systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) score at the time of BSLE diagnosis was 9 (3–23), suggesting the presence of moderate to severe SLE disease in these patients [ 5 ]. Interestingly, our patient presented a SLEDAI-2K of 22 and developed both severe renal compromise with nephrotic syndrome and urine sediment highly suggestive of lupus nephritis treated with mycophenolate mofetil and warm autoimmune hemolytic anemia requiring rituximab.…”

Section: Discussionmentioning

confidence: 99%

“…The use of steroids and other immunosuppressants such as methotrexate, azathioprine, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin is indicated in combination with dapsone when systemic symptoms of SLE are present [ 3 , 6 – 8 , 20 , 21 ]. In cases of dapsone contraindications, the literature suggests the use of steroids and immunosuppressant agents only [ 1 , 5 ]. There are two different dapsone contraindications: absolute and relative.…”

Section: Discussionmentioning

confidence: 99%

“…The correlation between BSLE and SLE activity remains controversial. Nonetheless, recent evidence shows that BSLE is rarely isolated and is usually associated with extracutaneous manifestations suggestive of severe disease [ 5 ]. Dapsone is considered first-line treatment.…”

Section: Introductionmentioning

confidence: 99%

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Bullous Lupus: An Atypical Case of Refractory Disease in a Patient with Sulfa Allergy

Guerrero

Miranda

Arevalo³

et al. 2020

Case Reports in Rheumatology

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Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous autoimmune disorder characterized by rapid, widespread vesiculobullous lesions in patients with Systemic Lupus Erythematosus (SLE). BSLE can present as the initial manifestation of SLE and may be a marker of severe disease. In this case report, we present a case of a 22-year-old African American woman with BSLE and impaired renal function with subsequent nephrotic range proteinuria concerning for lupus nephritis and autoimmune hemolytic anemia, refractory to systemic corticosteroids, immunoglobulin, and mycophenolate mofetil, requiring dapsone after careful desensitization due to prior history of angioedema with sulfa drugs. This case highlights the importance of the prompt recognition of BSLE as the initial manifestations of SLE and illustrates the association of BSLE with severe disease and the benefit of concomitant use of dapsone with corticosteroids and other immunosuppressant drugs, even in patients with a history of sulfa allergy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Bullous Lupus: An Atypical Case of Refractory Disease in a Patient with Sulfa Allergy

Guerrero

Miranda

Arevalo³

et al. 2020

Case Reports in Rheumatology

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“…Furthermore, dapsone may be discussed as a first-line agent in neutrophilic bullous lupus. Indeed, a recent literature review of 128 published cases found a response of 90% [88]. A low starting dose of 50 mg/day is recommended, then the dose could be increased to usually about 100 to 150 mg/day (maximum 400 mg/day or 2 mg/day/kg in children) [89].…”

Section: Dapsonementioning

confidence: 99%

Current Concepts and Future Approaches in the Treatment of Cutaneous Lupus Erythematosus: A Comprehensive Review

Chasset

Françès

2019

Drugs

Self Cite

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Standard treatment of cutaneous lupus erythematosus (CLE) includes preventive measures such as smoking cessation and photoprotection associated with topical therapies and antimalarial agents, which are recommended as first-line systemic treatment. In more severe disease, alternative therapeutic options include immunosuppressive and immunomodulatory drugs. Recently, the development of specific tools to assess CLE activity and the publication of European CLE guidelines have improved the management of CLE. Moreover, several biologic agents are currently studied specifically in CLE or in systemic lupus erythematosus with assessment of skin involvement and may be promising therapies. However, improvement of the management of CLE remains a major unmet need. In this review, we summarize current concepts in the management of CLE as well as future approaches for more targeted treatments. Key points:  Some targeted agents that have shown some promise in skin manifestations with systemic lupus erythematosus.  Future studies are needed specifically for cutaneous lupus erythematosus (CLE), with responses for each of the CLE subtypes reported separately.

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Immunobullous Diseases

Schmidt,

Groves

2024

Rook's Textbook of Dermatology

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The immunobullous disorders represent a group of conditions characterised by antibody‐mediated autoimmune responses against structural elements of the skin resulting in blistering of the skin and/or mucosae. Antibody targets include proteins in the hemidesmosomes basement membrane zone and superficial dermis (pemphigoid group), desmosomes (pemphigus group) and epidermal and tissue‐type transglutaminase (dermatitis herpetiformis). If left untreated, immunobullous disorders are associated with significant morbidity and mortality and thus prompt, accurate diagnosis and treatment are mandatory. Therapeutic options include corticosteroids, steroid‐sparing immunosuppressants such as azathioprine, mycophenolate mofetil and anti‐inflammatory agents such as dapsone and tetracyclines. In severe and/or refractory patients, intravenous immunoglobulin, immunoadsorption and anti‐B‐cell agents such as rituximab may be of benefit. Indeed, recently international and European guidelines have recommended the use of rituximab as first line treatment in moderate and severe pemphigus vulgaris and foliaceous.

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Clinical, histological, immunological presentations and outcomes of bullous systemic lupus erythematosus: 10 New cases and a literature review of 118 cases

Cited by 70 publications

References 73 publications

Bullous Lupus: An Atypical Case of Refractory Disease in a Patient with Sulfa Allergy

Bullous Lupus: An Atypical Case of Refractory Disease in a Patient with Sulfa Allergy

Current Concepts and Future Approaches in the Treatment of Cutaneous Lupus Erythematosus: A Comprehensive Review

Immunobullous Diseases

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