Porfiria cutánea tardía: Reporte de 5 casos

La PCT es la mas comun de las porfirias. Es una fotodermatosis que resulta de la deficiencia de la UPD, enzima perteneciente a la via de sintesis del hemo. Presentamos la evolucion de cinco casos de PCT; cuatro de PCT familiar y uno de PCT esporadica. En dos de los pacientes pertenecientes al grupo de PCT tipo II, encontramos como factor de riesgo, el consumo de alcohol, y en la paciente con PCT tipo I se detecto serologia positiva para VHC. Todos los pacientes fueron tratados con cloroquina y flebotomias repetidas. Rapidamente se detecto mejoria clinica y bioquimica. Se observo que la porfirinuria continuo en descenso aun luego de suspendida la terapeutica. Tres de los pacientes con PCT familiar persisten en remision clinica y con ausenciade recaidas tras mas de 10 anos de seguimiento. Aconsejamos en pacientes que padecen PCT la busqueda de factores asociados (VHC, HIV, genes de HH) y desencadenantes exogenos (consumo excesivo de alcohol, hierro en la dieta e ingesta de estrogenos) que de ser controlados o evitados, junto con el tratamiento oportuno, contribuyen a un satisfactorio control de la enfermedad.(AU) The porphyria cutanea tarda is the most frequent porphyria, it is a photodermatosis secondary to uroporphyrinogen decarboxylase deficiency; this enzyme belongs to the haem synthesis pathway. We present on this paper the evolution of five cases of PCT, four of them with familiar type and one of them sporadic type. In two patients belonging to PCT type II, we found alcohol addiction as a serious risk, while on the other patients PCT type I we found HCV positive serology. All patients were treated with chloroquine and phlebotomies. We could observe a good response not only clinical but biochemical. We could also see that the porphyrins urinary level continued descending once the drug was withdrew. Three of the patients with familiar PCT remains in clinical remission without any relapses in ten years of control. We advice all the patients that suffer PCT the detection of associated factors such as HCV, HIV, HH genes and the avoidance of the exogenous triggering factors such as excessive intake of alcohol, dietary iron and estrogen intake. Taking these advices into account, together with the correct treatment every patient can control this disease positively or satisfactorily.(AU)