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

• Published in: Seminars in arthritis and rheumatism Vol. 48; no. 1; pp. 83 - 89
• Main Authors: de Risi-Pugliese, Tullia, Cohen Aubart, Fleur, Haroche, Julien, Moguelet, Philippe, Grootenboer-Mignot, Sabine, Mathian, Alexis, Ingen-Housz-Oro, Saskia, Hie, Miguel, Wendremaire, Noémie, Aucouturier, Françoise, Lepelletier, François, Miyara, Makoto, Bader-Meunier, Brigitte, Rémy, Philippe, Fabien, Nicole, Francès, Camille, Barete, Stéphane, Amoura, Zahir
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.08.2018
• Subjects: Autoimmune blistering disease; Blisters; Bullous lupus erythematosus; Lupus; Vesiculobullous; ISN/RPS; LN; DH; EBA; SLICC; Autoimmune blistering disease; Bullous lupus erythematosus; SLE; BP; PNN; C7; BSLE; IB; CYP; DIF; Blisters; Vesiculobullous; Lupus; IIF; MMF; AZA; BMZ; ACR; IEM; SLEDAI-2K; SSS; AIBD; ANA; HCQ; TEN; Ig; ELISA
• ISSN: 0049-0172; 1532-866X; 1532-866X
• DOI: 10.1016/j.semarthrit.2017.11.003

Bullous systemic lupus erythematosus (BSLE) is a rare blistering condition associated with systemic lupus erythematosus (SLE). We conducted a multi-center retrospective study and literature review in order to describe the clinical, immunological, and histological presentations and outcomes of BSLE. The skin biopsies were centrally reviewed, and sera obtained during a flare of BSLE were analyzed for identification of circulating anti-basement membrane zone antibodies. Ten patients (all women, median age at SLE diagnosis of 22 years) were included, as well as 118 cases from a systematic review of the literature. Lupus nephritis was associated in 50% of the cases. BSLE presented as tensed bullae on normal or erythematous skin, predominantly localized on the trunk, arms, head, and neck. Urticarial lesions were associated in 31% of the cases, and mucous membrane involvement was seen in 51%. Histological analyses displayed subepidermal detachment, dermal infiltration of polynuclear neutrophils, alignment of these cells at the basal membrane zone and leukocytoclasis. The direct immunofluorescence was polymorphic, showing linear and/or granular deposits of IgG, IgA, IgM, and/or C3. Anti-type VII collagen antibodies were detected in 69% of cases. Dapsone was efficacious in 90% of cases. BSLE is rather an autoimmune neutrophilic blistering disease associated with SLE than a cutaneous manifestation and may be associated with active extra-cutaneous manifestations of SLE. Dapsone is the first-choice option.