Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins).

Saskia Ingen-Housz-Oro (1, 2) , Tu-Anh Duong (1) , Benoit Bensaid (3) , Nathalia Bellon (4) , Nicolas de Prost (5) , Dévy Lu (1) , Bénédicte Lebrun-Vignes (6) , Julie Gueudry (7) , Emilie Bequignon (8) , Karim Zaghbib (9) , Gérard Royer (10) , Audrey Colin (1) , Giao Do-Pham (11) , Christine Bodemer (4) , Nicolas Ortonne (12) , Annick Barbaud (13) , Laurence Fardet (1, 2) , Olivier Chosidow (1, 2) , Pierre Wolkenstein (1, 2)

1 Service de dermatologie [Mondor]
2 EpiDermE - Epidemiology in Dermatology and Evaluation in Therapeutics
3 Hôpital Edouard Herriot [CHU - HCL]
4 Service de dermatologie [CHU Necker]
5 Service de réanimation médicale
6 CHU Pitié-Salpêtrière [AP-HP]
7 Service d'ophtalmologie [Rouen]
8 Service d'ORL et de chirurgie cervico-faciale
9 Pôle de Psychiatrie [Hôpital Henri Mondor]
10 Hôpital Henri Mondor
11 CHI Créteil
12 Département de pathologie [Mondor]
13 CHU Tenon [AP-HP]

Epidermal necrolysis (EN) encompasses Stevens-Johnson syndrome (SJS, < 10% of the skin affected), Lyell syndrome (toxic epidermal necrolysis, TEN, with ≥30% of the skin affected) and an overlap syndrome (10 to 29% of the skin affected). These rare diseases are caused, in 85% of cases, by pharmacological treatments, with symptoms occurring 4 to 28 days after treatment initiation. Mortality is 20 to 25% during the acute phase, and almost all patients display disabling sequelae (mostly ocular impairment and psychological distress).The objective of this French national diagnosis and care protocol (protocole national de diagnostic et de soins; PNDS), based on a critical literature review and on a multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with EN. This PNDS, written by the French National Reference Center for Toxic Bullous Dermatoses was updated in 2017 ( https://www.has-sante.fr/portail/jcms/c_1012735/fr/necrolyse-epidermique-syndromes-de-stevens-johnson-et-de-lyell ). The cornerstone of the management of these patients during the acute phase is an immediate withdrawal of the responsible drug, patient management in a dermatology department, intensive care or burn units used to dealing with this disease, supportive care and close monitoring, the prevention and treatment of infections, and a multidisciplinary approach to sequelae. Based on published data, it is not currently possible to recommend any specific immunomodulatory treatment. Only the culprit drug and chemically similar molecules must be lifelong contraindicated.

Mots clés: Causality Background; Lyell syndrome; Intensive care; Drug reaction; Treatment; Management; Stevens-Johnson syndrome; Causality; Toxic epidermal necrolysis

Domaines: Dermatologie

Saskia Ingen-Housz-Oro, Tu-Anh Duong, Benoit Bensaid, Nathalia Bellon, Nicolas de Prost, et al.. Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins).. Orphanet Journal of Rare Diseases, 2018, 13 (1), pp.56. ⟨10.1186/s13023-018-0793-7⟩. ⟨hal-01975447⟩