Lupus (2017) 0, 1–7 journals.sagepub.com/home/lup PAPER A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients K Chanprapaph, S Sawatwarakul and V Vachiramon Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand 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 ery- thematosus 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 hemato- logic and renal impairment, in patients presenting with bullous systemic lupus erythematosus. Lupus (2017) 0, 1–7. Key words: Bullous systemic lupus erythematosus; hemolytic anemia; leukopenia; lupus erythematosus; lupus nephritis; lymphopenia Introduction Bullous systemic lupus erythematosus (BSLE) is an autoimmune subepidermal blistering eruption occurring rarely in patients with systemic lupus ery- thematosus (SLE). Cutaneous manifestations were observed in 59–85% of patients with SLE, however, less than 5% developed vesiculobullous lesions. 1 The reported incidence of BSLE is approximately 2–3% of all subepidermal autoimmune bullous skin diseases. 2 Clinically, patients present with widespread blistering arising on erythematous or normal skin and heal without scarring or milia. Subepidermal vesicles with predominant neutrophil infiltration are the classic histopathologic finding. Distinctive immunopathologic features are multiple immunoreactants (IgG, IgA, IgM and complement) along the basement membrane zone (BMZ). 1 The presence of circulating antibody on the dermal slide of the BMZ can be presented. 3 The major target antigen in patients with BSLE is the anchoring fibril component type VII collagen in the dermis. Cutaneous manifestations can occur in patients with active SLE, particularly lupus nephritis. 1 According to a literature review, there are limited data regarding BSLE and most of the published articles are case reports and case series. The aim Correspondence to: Kumutnart Chanprapaph, Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Rajathevi, Bangkok, 10400, Thailand. Email: kumutnartp@hotmail.com Received 26 November 2016; accepted 21 February 2017 ! The Author(s), 2017. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav 10.1177/0961203317699714