Porphyria cutanea tarda.
Porphyria cutanea tarda (PCT) is a metabolic disorder of haem biosynthesis caused by decreased activity of uroporphyrinogen decarboxylase. Porphyria cutanea tarda is manifest by fragility, erosions, bullae, milia and scars on sun-exposed skin. Excess porphyrins in the skin interact with light of approximately 400 nm-wavelength radiant energy, forming reactive oxygen species. Porphyria cutanea tarda is categorized as familial, acquired or toxic. Factors that may induce clinical expression of PCT in susceptible individuals include alcohol, oestrogen, iron, polyhalogenated compounds and viral infections. Porphyria cutanea tarda is associated with an increased incidence of the haemochromatosis gene. Treatments for PCT include withdrawal of aggravating factors, phlebotomy and oral antimalarial medications.
Bleasel
N R
NR
Department of Dermatology, Royal Melbourne Hospital, Victoria, Australia.
Varigos
G A
GA
D001315:Australia; D005260:Female; D006801:Humans; D008297:Male; D017119:Porphyria Cutanea Tarda / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D011379:Prognosis; D012307:Risk Factors