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1 Residente de Dermatologa, Universidad de Antioquia, Medelln-Colombia Correspondencia: [email protected] Mdica Dermatloga, Doctora en Ciencias Bsicas Biomdicas Inmunologa. Jefe Seccin de Dermatologa, Facultad de Medicina, Universidad de Antioquia, Grupo de Investi-
gacin Dermatologica GRID, Grupo de Estudio en Dermatologa
Recibido: agosto 18 de 2010 Aceptado: septiembre 14 de 2010
Inmunopatogenia del pngo vulgar y el pngo foliceo
scar Jairo Valencia Ocampo1, Margarita M. Velsquez - Lopera2
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Figura n. 2. Pngo foliceo. La piel aparece cubierta por escamas ycostras sobre una base eritematosa, algunas zonas denudadas. Las ampollas ci-das son poco aparentes. Foto Seccin de Dermatologa, Universidad de Antioquia
Figura n. 1. Pngo vulgar. Extensa denudacin de la piel y las mucosas.
Foto Seccin de Dermatologa, Universidad de Antioquia
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Figura n. 3. Estructura de los desmosomasLas molculas trasmembrana, desmoglenas y desmocolinas, se unen con queratinas del citoesqueleto mediante la interaccincon componentes intracelulares de la placa del desmosoma como desmoplaquina, placo lina, envoplaquina y periplaquina.Modicado de Herti M, Erning R, Veldam C. T cell control in autoimmune skin disorders. J Clin Invest 2006; 116: 115966
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Figura n. 4. Teora de compensacin de las desmoglenasLas Dsg-1 y Dsg-3 se encuentran distribuidas en diferentes proporciones en la piel y las mucosas. En PF la IgG es contra Dsg-1, el fenmenode acantlisis es subcrneo debido a que la cantidad de Dsg-3 no alcanza a compensar el bloqueo funcional de la Dsg-1; en las mucosas lacantidad de Dsg-3 es adecuada y suciente para mantener la adhesin. En el PV la mayora de los pacientes presentan IgG contra Dsg-1 y Dsg-3, el bloqueo funcional de ambas protenas induce acantlisis en piel y mucosas. Modicado de Stanley JR. Pemphigus. In: Wolff K, GoldsmithLA, Gilchrest BA, Paller AS, Leffell DJ. Fitzpatricks Dermatology in General Medicine. 7th ed. New York. Mc Graw Hill Medical, 2008. p 459-467
MucosaPiel
MucosaPiel
Anticuerpos anti-Dsg1
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Anticuerpos anti-Dsg3 y anti-Dsg1
13 3
1
1
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1
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Tabla n. 1. Autoantgenos en pngo vulgar y pngo foliceo
Tipo de pngo Antgenos principales Antgenos accesorios
Pngo vulgar Desmoglena-3Desmoglena-1
Desmoplaquina
Receptor de acetilcolina alfa-9Penfaxina y anexina
Desmoglena-4?
Pngo foliceo espordicoDesmoglena-1
Pngo foliceo endmico
(Colombia) Desmoglena-1
Desmoplaquina-1
Envoplaquina
PeriplaquinaProtena de 230 kD
Pngo foliceo endmico
(Brasil) Desmoglena-1
Desmoglena-3
Desmocolina-1, -3
Pngo foliceo endmico
(Per, Tnez)Desmoglena-1
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