Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins)
Abstract 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...
| Main Authors: | , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2018-04-01
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| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13023-018-0793-7 |
| _version_ | 1818898343719337984 |
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| author | Saskia Ingen-Housz-Oro Tu-Anh Duong Benoit Bensaid Nathalia Bellon Nicolas de Prost Dévy Lu Bénédicte Lebrun-Vignes Julie Gueudry Emilie Bequignon Karim Zaghbib Gérard Royer Audrey Colin Giao Do-Pham Christine Bodemer Nicolas Ortonne Annick Barbaud Laurence Fardet Olivier Chosidow Pierre Wolkenstein the French National Reference Center for Toxic Bullous Dermatoses |
| author_facet | Saskia Ingen-Housz-Oro Tu-Anh Duong Benoit Bensaid Nathalia Bellon Nicolas de Prost Dévy Lu Bénédicte Lebrun-Vignes Julie Gueudry Emilie Bequignon Karim Zaghbib Gérard Royer Audrey Colin Giao Do-Pham Christine Bodemer Nicolas Ortonne Annick Barbaud Laurence Fardet Olivier Chosidow Pierre Wolkenstein the French National Reference Center for Toxic Bullous Dermatoses |
| author_sort | Saskia Ingen-Housz-Oro |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-19T19:30:34Z |
| format | Article |
| id | doaj.art-396ecfb09a394a9fb44945ba62bf97bb |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-19T19:30:34Z |
| publishDate | 2018-04-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-396ecfb09a394a9fb44945ba62bf97bb2022-12-21T20:08:37ZengBMCOrphanet Journal of Rare Diseases1750-11722018-04-0113111110.1186/s13023-018-0793-7Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins)Saskia Ingen-Housz-Oro0Tu-Anh Duong1Benoit Bensaid2Nathalia Bellon3Nicolas de Prost4Dévy Lu5Bénédicte Lebrun-Vignes6Julie Gueudry7Emilie Bequignon8Karim Zaghbib9Gérard Royer10Audrey Colin11Giao Do-Pham12Christine Bodemer13Nicolas Ortonne14Annick Barbaud15Laurence Fardet16Olivier Chosidow17Pierre Wolkenstein18the French National Reference Center for Toxic Bullous DermatosesDermatology Department, AP-HP, Henri Mondor HospitalDermatology Department, AP-HP, Henri Mondor HospitalFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesDermatology Department, AP-HP, Henri Mondor HospitalFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesDermatology Department, AP-HP, Henri Mondor HospitalFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesFrench National Reference Center for Toxic Bullous DermatosesDermatology Department, AP-HP, Henri Mondor HospitalDermatology Department, AP-HP, Henri Mondor HospitalDermatology Department, AP-HP, Henri Mondor HospitalAbstract 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.http://link.springer.com/article/10.1186/s13023-018-0793-7Stevens-Johnson syndromeLyell syndromeToxic epidermal necrolysisManagementTreatmentIntensive care |
| spellingShingle | Saskia Ingen-Housz-Oro Tu-Anh Duong Benoit Bensaid Nathalia Bellon Nicolas de Prost Dévy Lu Bénédicte Lebrun-Vignes Julie Gueudry Emilie Bequignon Karim Zaghbib Gérard Royer Audrey Colin Giao Do-Pham Christine Bodemer Nicolas Ortonne Annick Barbaud Laurence Fardet Olivier Chosidow Pierre Wolkenstein the French National Reference Center for Toxic Bullous Dermatoses Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) Orphanet Journal of Rare Diseases Stevens-Johnson syndrome Lyell syndrome Toxic epidermal necrolysis Management Treatment Intensive care |
| title | Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) |
| title_full | Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) |
| title_fullStr | Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) |
| title_full_unstemmed | Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) |
| title_short | Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) |
| title_sort | epidermal necrolysis french national diagnosis and care protocol pnds protocole national de diagnostic et de soins |
| topic | Stevens-Johnson syndrome Lyell syndrome Toxic epidermal necrolysis Management Treatment Intensive care |
| url | http://link.springer.com/article/10.1186/s13023-018-0793-7 |
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