Toxic epidermic necrolysis: a high-mortality pathology
Toxic Epidermal Necrolysis (TEN) is a disease caused by delayed hypersensitivity reaction to drugs such as NSAIDs, allopurinol, carbamazepine and phenytoin. Its incidence: 0.5 - 1 cases per million persons, with a mortality of 30 - 60%. Erythematous macules-papules with blister of serous content and...
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| Formato: | Artículo revista |
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Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2019
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| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/26022 |
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I10-R10-article-26022 |
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ojs |
| institution |
Universidad Nacional de Córdoba |
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I-10 |
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R-10 |
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Revistas de la UNC |
| language |
Español |
| format |
Artículo revista |
| topic |
toxic epidermic necrolysis drug delayed hypersensitivity immunoglobulin necrolisis epidermica toxica hipersensibilidad retardada a drogas inmunoglobulina |
| spellingShingle |
toxic epidermic necrolysis drug delayed hypersensitivity immunoglobulin necrolisis epidermica toxica hipersensibilidad retardada a drogas inmunoglobulina Rojas, AB Poma Torres, S Salazar, V Ferreyra, L Vergottini, S Andreotti, M Chales, S Vergottini, JC Toxic epidermic necrolysis: a high-mortality pathology |
| topic_facet |
toxic epidermic necrolysis drug delayed hypersensitivity immunoglobulin necrolisis epidermica toxica hipersensibilidad retardada a drogas inmunoglobulina |
| author |
Rojas, AB Poma Torres, S Salazar, V Ferreyra, L Vergottini, S Andreotti, M Chales, S Vergottini, JC |
| author_facet |
Rojas, AB Poma Torres, S Salazar, V Ferreyra, L Vergottini, S Andreotti, M Chales, S Vergottini, JC |
| author_sort |
Rojas, AB |
| title |
Toxic epidermic necrolysis: a high-mortality pathology |
| title_short |
Toxic epidermic necrolysis: a high-mortality pathology |
| title_full |
Toxic epidermic necrolysis: a high-mortality pathology |
| title_fullStr |
Toxic epidermic necrolysis: a high-mortality pathology |
| title_full_unstemmed |
Toxic epidermic necrolysis: a high-mortality pathology |
| title_sort |
toxic epidermic necrolysis: a high-mortality pathology |
| description |
Toxic Epidermal Necrolysis (TEN) is a disease caused by delayed hypersensitivity reaction to drugs such as NSAIDs, allopurinol, carbamazepine and phenytoin. Its incidence: 0.5 - 1 cases per million persons, with a mortality of 30 - 60%. Erythematous macules-papules with blister of serous content and denudation ≥ 30% of body surface are observed on the skin. It affects the mucous membranes and is accompanied by fever, general discomfort, and myalgia, resulting in sepsis, acute renal failure, acute respiratory failure and Multiorgan Dysfunction (MOD). The prognosis is based on the SCORTEN Score, which assesses age ≥ 40 years, plasma urea ≥ 28 mg/dl, affected body surface area ≥10%, serum bicarbonate ≥ 20 mEq/L and plasma blood glucose 250 mg/dl. A SCORTEN of ≥ 5 points indicates a mortality > 90%. The pathogenesis is genetic, related to the HLA system, activation of CD8 + T lymphocytes, Natural Killer cells, FasL activation, perforin, granzyme B and granulosin, TNF α and If γ. The biopsy reveals epidermal necrosis with inflammatory infiltration and dermo-epidermal detachment. The treatment is based on the administration of Immunoglobulin.
Case presentation: 75 year old female patient with a history of chronic arterial hypertension and hypothyroidism. Religion: Jehovah's Witness. No allergic history. He was admitted to the ICU with a diagnosis of Hemorrhagic Stroke, requiring dexamethasone and phenytoin. At 3 weeks he manifested fever, general discomfort and the appearance of blisters erythematous lesions on the face, abdomen, back, both buttocks, thorax and armpits, and other ulcerated types in the mouth and labia majora. In addition, he developed acute respiratory failure, sepsis and MOD. Phenytoin was suspended. Skin biopsy showed epidermal necrosis with subdermal detachment, with perivascular mononuclear leukocyte infiltrate. The SCORTEN was 5 points. Due to the religious creed of the patient, treatment with Immunoglobulin was not initiated. Subsequently the patient obitiated.
The TEN is uncommon and high mortality, so the pharmacological choice must be careful. The diagnosis is based on skin biopsy processed with Hematoxylin/Eosin staining. Immunoglobulin treatment and critical support should be early. |
| publisher |
Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2019 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/26022 |
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2022-08-20T01:26:58Z |
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I10-R10-article-260222019-11-11T21:18:27Z Toxic epidermic necrolysis: a high-mortality pathology Necrolisis epidérmica toxica: una patología de alta mortalidad Rojas, AB Poma Torres, S Salazar, V Ferreyra, L Vergottini, S Andreotti, M Chales, S Vergottini, JC toxic epidermic necrolysis drug delayed hypersensitivity immunoglobulin necrolisis epidermica toxica hipersensibilidad retardada a drogas inmunoglobulina Toxic Epidermal Necrolysis (TEN) is a disease caused by delayed hypersensitivity reaction to drugs such as NSAIDs, allopurinol, carbamazepine and phenytoin. Its incidence: 0.5 - 1 cases per million persons, with a mortality of 30 - 60%. Erythematous macules-papules with blister of serous content and denudation ≥ 30% of body surface are observed on the skin. It affects the mucous membranes and is accompanied by fever, general discomfort, and myalgia, resulting in sepsis, acute renal failure, acute respiratory failure and Multiorgan Dysfunction (MOD). The prognosis is based on the SCORTEN Score, which assesses age ≥ 40 years, plasma urea ≥ 28 mg/dl, affected body surface area ≥10%, serum bicarbonate ≥ 20 mEq/L and plasma blood glucose 250 mg/dl. A SCORTEN of ≥ 5 points indicates a mortality > 90%. The pathogenesis is genetic, related to the HLA system, activation of CD8 + T lymphocytes, Natural Killer cells, FasL activation, perforin, granzyme B and granulosin, TNF α and If γ. The biopsy reveals epidermal necrosis with inflammatory infiltration and dermo-epidermal detachment. The treatment is based on the administration of Immunoglobulin. Case presentation: 75 year old female patient with a history of chronic arterial hypertension and hypothyroidism. Religion: Jehovah's Witness. No allergic history. He was admitted to the ICU with a diagnosis of Hemorrhagic Stroke, requiring dexamethasone and phenytoin. At 3 weeks he manifested fever, general discomfort and the appearance of blisters erythematous lesions on the face, abdomen, back, both buttocks, thorax and armpits, and other ulcerated types in the mouth and labia majora. In addition, he developed acute respiratory failure, sepsis and MOD. Phenytoin was suspended. Skin biopsy showed epidermal necrosis with subdermal detachment, with perivascular mononuclear leukocyte infiltrate. The SCORTEN was 5 points. Due to the religious creed of the patient, treatment with Immunoglobulin was not initiated. Subsequently the patient obitiated. The TEN is uncommon and high mortality, so the pharmacological choice must be careful. The diagnosis is based on skin biopsy processed with Hematoxylin/Eosin staining. Immunoglobulin treatment and critical support should be early. La Necrólisis Epidérmica Tóxica (NET) es una enfermedad por reacción de hipersensibilidad retardada a drogas como los AINES, allopurinol, carbamazepina y fenitoína. Su incidencia: 0,5 – 1 caso/millón de habitantes con una mortalidad del 30 - 60 %. En piel se observan máculas-pápulas eritematosas con ampollas de contenido seroso y una denudación ≥ 30 % de la superficie corporal. Afecta a las mucosas y se acompaña de fiebre, malestar general y mialgias, derivando en sepsis, fallo renal agudo, insuficiencia respiratoria aguda y disfunción multiorgánica (DMO). El pronóstico se basa en el Score SCORTEN, que evalúa la edad ≥ 40 años, la urea plasmática ≥ 28 mg/dl, la superficie corporal afectada ≥10 %, el bicarbonato sérico ≥ 20 mEq/L y la glucemia plasmática 250 mg/dl. Un SCORTEN de ≥ 5 ptos indica una mortalidad > 90 %. La patogénesis es genética, relacionada al sistema HLA, activación de Linfocitos T CD8+, células Natural Killer, activación del FasL, perforina, granzima B y granulosina, FNT α e If γ. La biopsia revela necrosis epidérmica con infiltración inflamatoria y despegamiento dermo-epidérmico. El tratamiento se basa en la administración de Inmunoglobulina. Presentación del caso: Paciente femenina de 75 años con Antecedentes de Hipertensión arterial crónica e hipotiroidismo. Religión: Testigo de Jehová. Sin antecedentes alérgicos. Ingresó a UTI con diagnóstico de Accidente Cerebrovascular Hemorrágico, requiriendo dexametasona y fenitoína. A las 3 semanas manifestó fiebre, malestar general y la aparición de lesiones eritematosas ampollares en cara, abdomen, dorso, ambos glúteos, tórax y axilas, y otras de tipo ulceradas en boca y labios mayores. Paralelamente desarrolló insuficiencia respiratoria aguda, sepsis y DMO. Se suspendió fenitoína. La biopsia de piel evidenció necrosis epidérmica con despegamiento subdérmico, con infiltrado leucocitario mononuclear perivascular. El SCORTEN fue de 5 ptos. Debido al credo religioso de la paciente, no se inició el tratamiento con Inmunoglobulina. Posteriormente la paciente obitó. La NET es infrecuente y de alta mortalidad, por lo que la elección farmacológica debe ser cuidadosa. El diagnóstico se basa en la biopsia de piel procesada con coloración de Hematoxilina/Eosina. El tratamiento con Inmunoglobulina y el soporte crítico deben ser precoces. Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2019-10-28 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/26022 Revista de la Facultad de Ciencias Médicas de Córdoba.; 2019: Suplemento JIC XX Revista de la Facultad de Ciencias Médicas de Córdoba; 2019: Suplemento JIC XX Revista da Faculdade de Ciências Médicas de Córdoba; 2019: Suplemento JIC XX 1853-0605 0014-6722 spa https://revistas.unc.edu.ar/index.php/med/article/view/26022/27886 Derechos de autor 2019 Universidad Nacional de Córdoba |