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Vol. 104. Num. 7.September 2013
Pages 541-644
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Vol. 104. Num. 7.September 2013
Pages 541-644
Novelties in dermatology
DOI: 10.1016/j.adengl.2012.11.020
Advances in the Diagnosis and Treatment of Tumor Necrosis Factor Receptor–Associated Periodic Syndrome
Novedades en el diagnóstico y tratamiento del síndrome periódico asociado al receptor del factor de necrosis tumoral
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L. Aguado-Gil??
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laguado@unav.es

Corresponding Author.
, I. Irarrazaval-Armendáriz, M. Pretel-Irazabal
Departamento de Dermatología, Clínica Universidad de Navarra, Pamplona, Spain
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Tables (2)
Table 1. Classification of Hereditary Systemic Autoinflammatory Diseases.
Table 2. Diagnostic Criteria for Tumor Necrosis Factor Receptor-Associated Periodic Syndrome.
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Abstract

Tumor necrosis factor receptor–associated periodic syndrome (TRAPS) is a rare autosomal dominant disease included in the group of autoinflammatory syndromes. It is characterized by recurrent episodes of fever and inflammation in different regions of the body. The main clinical manifestations are myalgia, migratory erythematous rash, periorbital edema, and abdominal pain. The diagnosis is reached using gene analysis and prognosis depends on the appearance of amyloidosis secondary to the recurrent episodes of inflammation. Tumor necrosis factor inhibitors and corticosteroids are the most widely used treatments. In recent years, significant advances have been made in the diagnosis and treatment of TRAPS, thanks to a better understanding of its pathogenesis. Dermatologists must be aware that the skin manifestations of TRAPS are particularly important, as they are often diagnostic.

Keywords:
Autoinflammatory diseases
Tumor necrosis factor receptor
Syndrome
Tumor necrosis factor receptor-associated periodic syndrome
Resumen

El síndrome periódico asociado al receptor del factor de necrosis tumoral (TRAPS) es una rara enfermedad autosómica dominante que forma parte de los síndromes autoinflamatorios. Se caracteriza por episodios recurrentes de fiebre e inflamación en distintos sitios del organismo, siendo sus principales manifestaciones: las mialgias, el exantema eritematoso migratorio, el edema periorbitario y el dolor abdominal. El diagnóstico se realiza mediante el análisis genético y su pronóstico está determinado por el desarrollo de amiloidosis, secundaria a los episodios inflamatorios repetidos. Los tratamientos más utilizados son los corticoides y los inhibidores del TNF. Durante los últimos años, gracias a un mayor conocimiento de su patogénesis, se han logrado importantes avances en su diagnóstico y tratamiento. Como dermatólogos es importante tener en cuenta que las manifestaciones cutáneas son particularmente importantes en el TRAPS, ya que muchas veces guían al clínico hacia su correcto diagnóstico.

Palabras clave:
Enfermedades autoinflamatorias
Receptor del factor de necrosis tumoral
Síndrome
Síndrome periódico asociado al receptor del factor de necrosis tumoral

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