Triggering factors in Porphyria Cutanea Tarda in Argentina

MELITO, VA; ROSSETTI, MV; BATLLE, A; PARERA, VE

Mar del Plata

Congreso; LXI REUNION CIENTIFICA ANUAL DE LA SOCIEDAD ARGENTINA DE INVESTIGACION CLINICA (SAIC); 2016

SSOCIEDAD ARGENTINA DE INVESTIGACION CLINICA (SAIC)

Porphyria Cutanea Tarda (PCT), a metabolic rare disease, is produced by reduced activity of Uroporphyrinogen Decarboxylase. Accumulation of highly carboxylated porphyrins in liver and plasma, mainly excreted by urine, are responsible of cutaneous damage: skin photosensitivity, blistering on exposed areas, fragility, hyperpigmentation and hyperthricosis. There are two principal types : Type I or A-PCT (acquired 75-80 %) and Type II or H-PCT (hereditary). It is a pharmacogenetic disease triggered by hepatotoxic drugs and hepatothropic virus. The relationship men/women is 4:1 in overt PCT. The aim of this work is to analyze the precipitating agents in young women. We studied 80 women up to 40 years old without HBV, HCV or HIV infection. PCT diagnosis was made by fluorometric measure of plasma porphyrin index (PPI), total urinary porphyrins (TUP) by ion exchange chromatography and porphyrin profile by thin layer chromatography (CRO). Mean age of PCT manifestation (29.0±6.4) was significantly lower (p