Canadian Medical Association journal · 1965
ACQUIRED PORPHYRIA CUTANEA TARDA: REPORT OF A CASE SUCCESSFULLY TREATED BY PHLEBOTOMY.
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KOVAL A, DANBY C W, PETERMANN H
Аннотация
CURRENTLY, THE PORPHYRIAS ARE CLASSIFIED IN FOUR MAIN GROUPS: congenital porphyria, acute intermittent porphyria, porphyria cutanea tarda hereditaria, and porphyria cutanea tarda symptomatica. The acquired form of porphyria (porphyria cutanea tarda symptomatica) occurs in older males and is nearly always associated with chronic alcoholism and hepatic cirrhosis. The main clinical changes are dermatological, with excessive skin fragility and photosensitivity resulting in erosions and bullae. Biochemically, high levels of uroporphyrin are found in the urine and stools. Treatment to date has been symptomatic and usually unsuccessful.A case of porphyria cutanea tarda symptomatica is presented showing dramatic improvement of both the skin lesions and porphyrin levels in urine and blood following repeated phlebotomy.Possible mechanisms of action of phlebotomy on porphyria cutanea tarda symptomatica are discussed.
Предметные рубрики MeSH
AlcoholismBiochemical PhenomenaBiochemistryBlisterBloodlettingBody FluidsDrug TherapyFecesHemoglobinometryHumansIronLiver CirrhosisMalePhlebotomyPhotosensitivity DisordersPorphyria Cutanea TardaPorphyria, Acute IntermittentPorphyria, ErythropoieticPorphyria, VariegatePorphyriasPorphyrinsSkin DiseasesUrineUroporphyrins