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      Bullous systemic lupus erythematosus: exceptional and challenging presentation

      case-report

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          Abstract

          Abstract Vesiculobullous lesions can be developed by less than 5% of patients with systemic lupus erythematosus (SLE). Among these lesions, bullous systemic lupus erythematosus (BSLE) is one of the rarest and most challenging cutaneous presentations of the disease. Its frequency in the largest series of 5149 patients with cutaneous lupus was 0.19%, and the majority of cases are reported as sporadic observation or small series not exceeding ten cases. We report the original observation of BSLE revealing SLE in 32-year-old Tunisian Woman. This atypical presentation of cutaneous lupus deserves to be known by clinicians and discussed in front of any bullous dermatosis, particularly in young woman.

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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.

          Tullia de Risi-Pugliese, Fleur Cohen Aubart, Julien Haroche … (2018)
          Bullous systemic lupus erythematosus (BSLE) is a rare blistering condition associated with systemic lupus erythematosus (SLE).
          Article 0 comments Cited 27 times – based on 0 reviews     Review now
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            Treatment of Bullous Systemic Lupus Erythematosus

            Lihua Duan, Liying Chen, Shan Zhong … (2015)
            Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated vesiculobullous disease in patients with SLE. Autoimmunity in BSLE is characterized by the presence of circulating anti-type VII collagen antibodies. BSLE patients often present with multiple, tense, clear fluid-filled vesicles and bullae overlying erythematous edematous plaques. Skin biopsy from BSLE patients shows subepidermal bullae with numerous neutrophils and only occasional eosinophils. Furthermore, immunofluorescence examination showed linear deposition of lgG, lgA, C3, and C1q along the basement membrane zone. BSLE patients with corticosteroids treatment constantly do not receive a marked improvement, while dapsone generally dramatically improved the skin condition. Recently, it has been reported that quite a few cases of BSLE were successfully treated with other immune suppressive drugs. Therefore, a comprehensive review of the treatment of BSLE would be beneficial to cure the disease.
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              A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients.

              K. Chanprapaph, S Sawatwarakul, V. Vachiramon (2017)
              Objective The aim of this study was to investigate the clinical features, laboratory findings, systemic manifestations, treatment and outcome of patients with bullous systemic lupus erythematosus in a tertiary care center in Thailand. Methods We performed a retrospective review from 2002 to 2014 of all patients who fulfilled the diagnostic criteria for bullous systemic lupus erythematosus to evaluate for the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence pattern, serological abnormalities, internal organ involvement, treatments and outcome. Results Among 5149 patients with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 15 developed vesiculobullous lesions. Ten patients had validation of the diagnosis of bullous systemic lupus erythematosus, accounting for 0.19%. Bullous systemic lupus erythematosus occurred after the diagnosis of systemic lupus erythematosus in six patients with a median onset of 2.5 months (0-89). Four out of 10 patients developed bullous systemic lupus erythematosus simultaneously with systemic lupus erythematosus. Hematologic abnormalities and renal involvement were found in 100% and 90%, respectively. Polyarthritis (40%) and serositis (40%) were less frequently seen. Systemic corticosteroids, immunosuppressants, antimalarials and dapsone offered resolution of cutaneous lesions. Conclusion Bullous systemic lupus erythematosus is an uncommon presentation of systemic lupus erythematosus. Blistering can occur following or simultaneously with established systemic lupus erythematosus. We propose that clinicians should carefully search for systemic involvement, especially hematologic and renal impairment, in patients presenting with bullous systemic lupus erythematosus.
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                Author and article information

                Journal
                Journal ID (publisher-id): ijm
                Title: Iberoamerican Journal of Medicine
                Abbreviated Title: Iberoam J Med
                Publisher: Hospital San Pedro (Logroño, La Rioja, Spain )
                ISSN (Print): 2695-5075
                ISSN (Electronic): 2695-5075
                Publication date (Print and electronic): 2020
                Volume: 2
                Issue: 4
                Pages: 388-390
                Affiliations
                [2] Sfax orgnameUniversity of Sfax orgdiv1Sfax Faculty of Medicine Túnez
                [5] Sfax orgnameUniversity of Sfax orgdiv1Sfax Faculty of Medicine Túnez
                [1] Gabes orgnameMilitary Hospital of Gabes orgdiv1Department of Internal Medicine Túnez
                [3] Gabes orgnameMilitary Hospital of Gabes orgdiv1Department of Internal Medicine Túnez
                [4] Gabes orgnameMilitary Hospital of Gabes orgdiv1Department of Internal Medicine Túnez
                Article
                Publisher ID: S2695-50752020000400022 Publisher ID: S2695-5075(20)00200400022
                DOI: 10.5281/zenodo.3965555
                SO-VID: 2fad21fb-c61a-4c77-81d6-30abae7eae17
                License:

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                Date accepted : 29 July 2020
                Date received : 09 July 2020
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 13, Pages: 3
                Product

                SciELO Spain

                Categories
                Subject: Case Report

                Keywords: Systemic lupus erythematosus,Cutaneous lupus,Bullous systemic lupus erythematosus,Skin
                Data availability:
                Keywords: Systemic lupus erythematosus, Cutaneous lupus, Bullous systemic lupus erythematosus, Skin

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