Psoriasis pustulosa generalizada: de la inmunopatogénesis a la clínica

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Artículo de revisión
Rev Asoc Colomb Dermatol. 2017; 25: 2 (abril-junio), 130-142

Psoriasis pustulosa generalizada: de la inmunopatogénesis a la clínica

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Autor(es): 
Andrea Castaño
andreacv29@gmail.com y mmvelasquez@yahoo.com
Médica, residente de Dermatología, Universidad de Antioquia, Medellín, Colombia
Margarita María Velásquez
Médica dermatóloga, Ph.D. en Ciencias Básicas Biomédicas; docente de Dermatología, Universidad de Antioquia; Centro de Investigaciones Dermatológicas CIDERM, Medellín, Colombia.
Resumen: 

El síndrome DITRA (Interleukin-36-Receptor Antagonist Deficiency) es una enfermedad autoinflamatoria debida a mutaciones del gen IL36RN que producen deficiencia del antagonista del receptor de la IL-36, lo que induce una cascada inflamatoria que lleva a un cuadro clínico grave de psoriasis pustulosa generalizada. Ante desencadenantes externos, como los componentes de agentes infecciosos que son activadores de los receptores de inmunidad innata, denominados PAMP (Pathogen-Associated Molecular Patterns), o los asociados a estrés celular, llamados DAMP (Damage-Associated Molecular Patterns), se activa el receptor con gran homología a los TLR (Toll-Like Receptors) y se amplifica a través del receptor de la IL-36, que en ausencia de su antagonista, conlleva a mayor activación celular en el principal órgano blanco, que es la piel.
El síndrome DITRA debe sospecharse en todo paciente con psoriasis pustulosa generalizada grave, para enfocar apropiadamente su tratamiento.
 

Palabras clave: 
deficiencia del antagonista del receptor de la interleucina 36; interleucina 36; receptor de interleucina 36; proteína IL-36Ra; proteína L-36ª; proteína IL-36B; psoriasis pustulosa, IL36RN; enfermedades autoinflamatorias hereditarias.

Summary: 

The DITRA syndrome is an autoinflammatory disease due to mutations in the IL36RN gene causing deficiency of receptor´s antagonist of IL-36, leading to an inflammatory cascade that causes clinical symptoms of generalized pustular psoriasis. External triggers such as components of infectious agents, which are activators of innate immune receptors, called PAMP (Pathogen-Associated Molecular Patterns) or associated with cellular stress (DAMP: Damage-Associated Molecular Patterns), activate the receptor highly homologous to the TLR (toll-like receptors) (1), and amplified through the receptor to IL-36, in the absence of its antagonist leads to increased cell activation in the main target organ, which is the skin. 
The DITRA syndrome should be suspected in any patient with severe generalized pustular psoriasis, to properly focus their treatment.
 

Key words: 
Deficiency of interleukin-36 receptor antagonist, interleukin 36, interleukin-36 receptor, IL-36RN protein, IL-36A protein, IL-36B protein, pustulosis psoriasis, IL36RN, hereditary autoinflammatory diseases.
PDF del articulo
Tabla 1. Citocinas IL-36, sus antagonistas y su receptor. MDCs (células dendríticas derivadas de monocitos), PDCs (células dendríticas plasmocitoides), BMDCs (células dendríticas derivadas de la médula ósea)
Tabla 1. Citocinas IL-36, sus antagonistas y su...
Figura 1. Hallazgos clínicos de psoriasis pustular generalizada. Piel intensamente inflamada con placas extensas de eritema y pústulas superpuestas, con abundante descamación generalizada, localizadas en tórax y extremidades. Fotos Sección de Dermatología
Figura 1. Hallazgos clínicos de psoriasis pustul...
Tabla 2. Mutaciones conocidas en IL-36RN y las poblaciones donde se han encontrado.
Tabla 2. Mutaciones conocidas en IL-36RN y las p...
Tabla 3. Evidencia experimental del papel de IL-36 en el síndrome DITRA
Tabla 3. Evidencia experimental del papel de IL-...

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