Titre :

Cutaneous Involvement in Catastrophic Antiphospholipid Syndrome in a Multicenter Cohort of 65 Patients.

Auteur(s) :

Dupré, Anastasia [Auteur]
Morel, Nathalie [Auteur]
Hôpital Cochin [AP-HP]
Yelnik, Cécile [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Moguelet, Philippe [Auteur]
CHU Tenon [AP-HP]
Le Guern, Véronique [Auteur]
Hôpital Cochin [AP-HP]
Stammler, Romain [Auteur]
Nguyen, Yann [Auteur]
Hôpital Cochin [AP-HP]
Paule, Romain [Auteur]
Hôpital Foch [Suresnes]
Dufrost, Virginie [Auteur]
Centre Hospitalier Régional Universitaire de Nancy [CHRU Nancy]
Ackermann, Felix [Auteur]
Hôpital Foch [Suresnes]
Benhamou, Ygal [Auteur]
Endothélium, valvulopathies et insuffisance cardiaque [EnVI]
Godeau, Bertrand [Auteur]
Hôpital Henri Mondor
Lambert, Marc [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Duffau, Pierre [Auteur]
Université de Bordeaux [UB]
Mekinian, Arsène [Auteur]
Immunologie - Immunopathologie - Immunothérapie [CHU Pitié Salpêtrière] [I3]
Saadoun, David [Auteur]
CHU Pitié-Salpêtrière [AP-HP]
Mouthon, Luc [Auteur]
Hôpital Cochin [AP-HP]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Maillard, Hélène [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Levesque, Hervé [Auteur]
Endothélium, valvulopathies et insuffisance cardiaque [EnVI]
Université de Rouen Normandie [UNIROUEN]
Morell-Dubois, Sandrine [Auteur]
Hôpital Claude Huriez [Lille]
Leroux, Gaëlle [Auteur]
CHU Pitié-Salpêtrière [AP-HP]
Piette, Jean-Charles [Auteur]
CHU Pitié-Salpêtrière [AP-HP]
Chasset, François [Auteur]
CHU Tenon [AP-HP]
Costedoat-Chalumeau, Nathalie [Auteur]
Centre for Research in Epidemiology and Statistics | Centre de Recherche Épidémiologie et Statistiques [CRESS (U1153 / UMR_A 1125)]

Titre de la revue :

JAMA Dermatology

Nom court de la revue :

JAMA Dermatol

Numéro :

159

Pagination :

62-67

Date de publication :

2022-12-09

ISSN :

2168-6084

Discipline(s) HAL :

Sciences du Vivant [q-bio]

Résumé en anglais : [en]

Importance Catastrophic antiphospholipid syndrome (CAPS) is a severe, rare complication of antiphospholipid syndrome (APS), but cutaneous involvement has not yet been adequately described. Objective To describe cutaneous ...
Lire la suite >Importance Catastrophic antiphospholipid syndrome (CAPS) is a severe, rare complication of antiphospholipid syndrome (APS), but cutaneous involvement has not yet been adequately described. Objective To describe cutaneous involvement during CAPS, its clinical and pathological features, and outcomes. Design, Setting, and Participants This cohort study was a retrospective analysis of patients included in the French multicenter APS/systemic lupus erythematosus register (ClinicalTrials.gov: NCT02782039) by December 2020. All patients meeting the revised international classification criteria for CAPS were included, and patients with cutaneous manifestations were analyzed more specifically. Main Outcomes and Measures Clinical and pathological data as well as course and outcome in patients with cutaneous involvement during CAPS were collected and compared with those in the register without cutaneous involvement. Results Among 120 patients with at least 1 CAPS episode, the 65 (54%) with skin involvement (43 [66%] women; median [range] age, 31 [12-69] years) were analyzed. Catastrophic antiphospholipid syndrome was the first APS manifestation for 21 of 60 (35%) patients with available data. The main lesions were recent-onset or newly worsened livedo racemosa (n = 29, 45%), necrotic and/or ulcerated lesions (n = 27, 42%), subungual splinter hemorrhages (n = 19, 29%), apparent distal inflammatory edema (reddened and warm hands, feet, or face) (n = 15, 23%), and/or vascular purpura (n = 9, 14%). Sixteen biopsies performed during CAPS episodes were reviewed and showed microthrombi of dermal capillaries in 15 patients (94%). These lesions healed without sequelae in slightly more than 90% (58 of 64) of patients. Patients with cutaneous involvement showed a trend toward more frequent histologically proven CAPS (37% vs 24%, P = .16) than those without such involvement, while mortality did not differ significantly between the groups (respectively, 5% vs 9%, P = .47). Conclusions and Relevance In this cohort study, half the patients with CAPS showed cutaneous involvement, with a wide spectrum of clinical presentations, including distal inflammatory edema. Skin biopsies confirmed the diagnosis in all but 1 biopsied patient.Lire moins >

Langue :

Anglais

Audience :

Internationale

Vulgarisation :

Non

Établissement(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Date de dépôt :

2024-01-11T23:55:58Z
2024-03-11T16:10:19Z