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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
Las manifestaciones orales y gastrointestinales de la epidermolisis bullosa son las siguientes:
S P Travis1, J A McGrath, A J Turnbull
1Gastrointestinal Laboratory, St John's Institute of Dermatology, London, UK.
Lancet (London, England)
|December 19, 1992
Resumen
La epidermolisis bullosa (EB) afecta con frecuencia la boca y el ano, lo que lleva a disfagia y desnutrición, particularmente en subtipos graves como la EB distrófica recessiva. La intervención temprana y la atención especializada son cruciales para el manejo de estos síntomas debilitantes en pacientes con EB.
Área de la Ciencia:
- Dermatología Dermatología dermatología.
- Genética La genética.
- Gastroenterología y Gastroenterología.
Sus antecedentes:
- Epidermolysis bullosa (EB) abarca un grupo de enfermedades de la piel con ampollas genéticas raras.
- El involucramiento oromucosal y anal son complicaciones conocidas de ciertos subtipos de EB, pero su frecuencia precisa sigue siendo poco documentada.
- Estas complicaciones afectan significativamente la nutrición del paciente y la calidad de vida.
Objetivo del estudio:
- Para determinar la frecuencia de la intervención oral, esofágica y anal en pacientes con diversas formas de epiderolisis bullosa.
- Investigar la asociación entre los subtipos específicos de EB y la aparición de disfagia, microstomía y lesiones anales.
- Para resaltar los desafíos nutricionales y de gestión planteados por estas complicaciones.
Principales métodos:
- Un estudio retrospectivo de 246 pacientes diagnosticados con diferentes tipos de epiderolisis bullosa.
- Recopilación de datos sobre la presencia y la gravedad de las manifestaciones orales, esofágicas y anales.
- Análisis de la frecuencia de estas complicaciones en todos los subtipos de EB, incluida la EB distrófica recesiva, la EB distrófica dominante, la EB de unión y la EB simplex.
Principales resultados:
- La disfagia ocurrió en el 76% de los pacientes con EB distrófica recessiva, el 20% de los pacientes con EB distrófica dominante, el 15% de los pacientes con EB de unión y el 2% de los pacientes con EB simplex.
- Las adherencias linguales y la microstomía eran exclusivas de la EB distrófica, siendo ocho veces más prevalentes en las formas recesivas que en las dominantes.
- Se observaron ampollas anales que contribuyen al estreñimiento y la desnutrición.
Conclusiones:
- Las complicaciones orales y anales son frecuentes en la epidermolisis distrófica y en la epidermolisis bulosa de unión, lo que afecta significativamente el estado nutricional.
- La epidermolisis distrófica recesiva bullosa presenta una mayor carga de complicaciones orofaríngeas y potencialmente anales.
- La atención especializada multidisciplinaria es esencial para manejar las complejas necesidades de los pacientes con EB con estas manifestaciones.
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