Tullia de Risi-Pugliese1, Fleur Cohen Aubart2, Julien Haroche3, Philippe Moguelet4, Sabine Grootenboer-Mignot5, Alexis Mathian1, Saskia Ingen-Housz-Oro6, Miguel Hie1, Noémie Wendremaire5, Françoise Aucouturier7, François Lepelletier8, Makoto Miyara9, Brigitte Bader-Meunier10, Philippe Rémy11, Nicole Fabien12, Camille Francès13, Stéphane Barete14, Zahir Amoura3. 1. AP-HP, Service de Médecine Interne 2, Centre National de Référence des Maladies Systémiques Rares, Lupus et Syndrome des Anticorps antiphospholipides, Institut e3m, Groupe Hospitalier Pitié-Salpêtrière, Paris 75013, France. 2. AP-HP, Service de Médecine Interne 2, Centre National de Référence des Maladies Systémiques Rares, Lupus et Syndrome des Anticorps antiphospholipides, Institut e3m, Groupe Hospitalier Pitié-Salpêtrière, Paris 75013, France; Université Paris VI Pierre et Marie Curie, Sorbonnes Universités, Paris 75013, France. Electronic address: fleur.cohen@aphp.fr. 3. AP-HP, Service de Médecine Interne 2, Centre National de Référence des Maladies Systémiques Rares, Lupus et Syndrome des Anticorps antiphospholipides, Institut e3m, Groupe Hospitalier Pitié-Salpêtrière, Paris 75013, France; Université Paris VI Pierre et Marie Curie, Sorbonnes Universités, Paris 75013, France. 4. AP-HP, Service d'Anatomo-Pathologie, Hôpital Tenon, Paris 75020, France. 5. AP-HP, Laboratoire d'immunologie, Hôpital Bichat, Paris 75018, France. 6. AP-HP, Service de dermatologie, Hôpital Henri Mondor, Créteil 94000, France. 7. AP-HP, Laboratoire d'immunochimie, Hôpital Saint-Louis, Paris 75010, France. 8. AP-HP, Service d'Anatomo-Pathologie, Groupe Hospitalier Pitié-Salpêtrière, Paris 75013, France. 9. AP-HP, Service de Médecine Interne 2, Centre National de Référence des Maladies Systémiques Rares, Lupus et Syndrome des Anticorps antiphospholipides, Institut e3m, Groupe Hospitalier Pitié-Salpêtrière, Paris 75013, France; AP-HP, Département d'immunochimie, Groupe Hospitalier Pitié-Salpêtrière, Paris 75013, France. 10. AP-HP, Service d'Immunologie Pédiatrique, Hôpital Necker, Paris 75015, France. 11. AP-HP, Service de néphrologie, Hôpital Henri Mondor, Créteil 94000, France. 12. Laboratoire d'immunologie, Hôpital Lyon Sud, Lyon 49000, France. 13. Université Paris VI Pierre et Marie Curie, Sorbonnes Universités, Paris 75013, France; AP-HP, Service de Dermatologie-Allergologie, Hôpital Tenon, Paris 75020, France. 14. AP-HP, Service de Dermatologie-Allergologie, Hôpital Tenon, Paris 75020, France; AP-HP, UF Dermatologie, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
Abstract
BACKGROUND: Bullous systemic lupus erythematosus (BSLE) is a rare blistering condition associated with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.
BACKGROUND: Bullous systemic lupus erythematosus (BSLE) is a rare blistering condition associated with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.
Authors: Alice Verdelli; Alberto Corrà; Elena Biancamaria Mariotti; Cristina Aimo; Valentina Ruffo di Calabria; Walter Volpi; Lavinia Quintarelli; Marzia Caproni Journal: Front Med (Lausanne) Date: 2022-09-23