title: A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients.
abstract: 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.
authors.affiliation: Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: K
authors.initials: K
authors.lastname: Chanprapaph
authors.affiliation: Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: S
authors.initials: S
authors.lastname: Sawatwarakul
authors.affiliation: Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: V
authors.initials: V
authors.lastname: Vachiramon
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001168:Arthritis / Q000453:epidemiology / Q000209:etiology; D002648:Child; D005260:Female; D006801:Humans; D008178:Lupus Erythematosus, Cutaneous / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D008875:Middle Aged; D012189:Retrospective Studies; D012700:Serositis / Q000453:epidemiology / Q000209:etiology; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D013785:Thailand; D013997:Time Factors; D055815:Young Adult
keywords: Bullous systemic lupus erythematosus; hemolytic anemia; leukopenia; lupus erythematosus; lupus nephritis; lymphopenia