title: Blisters and Loss of Epidermis in Patients With Lupus Erythematosus: A Clinicopathological Study of 22 Patients.
abstract: The nosology of bullous lesions or equivalents (vesicles, erosions, and crusts) in patients with lupus erythematosus (LE) is rarely addressed.The primary aim of this study was to draw up a precise phenotypic inventory of such skin lesions; the secondary objective was to assess a potential relationship between the different types of loss of epidermis and extracutaneous lupus manifestations.We conducted a retrospective multicenter study including 22 patients with definite LE and bullous lesions or equivalents. All biopsies were reviewed. Patients were recruited in the dermatology departments of 6 centers. Patients were included if they met the diagnosis of systemic LE according to American College of Rheumatology and/or Systemic Lupus International Collaborating Clinics criteria or diagnosis of cutaneous LE based on classic clinical criteria and/or histological ascertainment of LE. Patients were recruited through clinician's memory and photographic collections.Three clinico-pathological patterns could be individualized. First, toxic epidermal necrolysis (TEN)-like, sheet-like, skin detachment; sun-exposure, mild mucosal involvement, and dermal mucin deposition allow differential diagnosis with classical Lyell syndrome. Second, vesiculo-bullae and/or crusting occurring on typical lesions of subacute cutaneous lupus erythematosus or chronic cutaneous lupus erythematosus. Third, tense vesicles and/or blisters with an underlying neutrophilic dermatosis and a usual response to dapsone.A careful analysis of 22 LE patients with epidermal detachment reveals 2 main pathomechanisms: a classic LE interface dermatitis, which can be hyperacute and lead to TEN-like skin detachment; and a neutrophilic dermatosis, with tense vesicles and/or blisters, including classic bullous LE.
authors.affiliation: From the Faculté de Médecine, Université de Strasbourg; Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg (CM-D, DL); Faculté de Médecine, Université de Montpellier; Département de Dermatologie, Hôpital Saint Eloi, CHRU Montpellier, Montpellier (DB); Faculté de Médecine, Université Pierre-et-Marie-Curie Paris-VI, Service de Dermatologie Hôpital Tenon, Paris (CF); Faculté de Médecine, Université Claude Bernard Lyon 1, Service de Dermatologie, CHU Lyon Sud, Pierre-Bénite (NP, SD); and Service de Dermatologie-Médecine interne, CHU de Pointe-à-Pitre, Pointe-à-Pitre, Guadeloupe, (NC) France.
authors.forename: Carine
authors.initials: C
authors.lastname: Merklen-Djafri
authors.forename: Didier
authors.initials: D
authors.lastname: Bessis
authors.forename: Camille
authors.initials: C
authors.lastname: Frances
authors.forename: Nicolas
authors.initials: N
authors.lastname: Poulalhon
authors.forename: Sébastien
authors.initials: S
authors.lastname: Debarbieux
authors.forename: Nadège
authors.initials: N
authors.lastname: Cordel
authors.forename: Dan
authors.initials: D
authors.lastname: Lipsker
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D001768:Blister / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D002648:Child; D004817:Epidermis / Q000276:immunology / Q000473:pathology*; D005260:Female; D006801:Humans; D008178:Lupus Erythematosus, Cutaneous / Q000276:immunology / Q000473:pathology*; D008179:Lupus Erythematosus, Discoid / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult