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Porphyrias are metabolic disorders of heme synthesis . Partial enzymic ____________________ result in excessive
accumulation and excretion of 5 - aminolevulinic acid , porphobilinogen , and / or porphyrins . ____________________
cutanea tarda ( PCT ) is the most common of the porphyrias ____________________ North America and Europe . First
described by Waldenström in 1937 , this ____________________ disorder is caused by a deficiency of
uroporphyrinogen decarboxylase , an ____________________ in heme biosynthesis . Porphyrins accumulate in the
liver , are ____________________ in plasma , and are excessively excreted in the ____________________ . Exposure of patients with
porphyria cutanea tarda to sunlight results in ____________________ skin fragility , vesicles , bullae , hypertrichosis ,
hyperpigmentation , sclerodermoid ____________________ , dystrophic calcification , milia , and scarring in a
photodistribution . Porphyria cutanea tarda ____________________ be inherited or acquired . Treatment options include
phlebotomy and ____________________ medications .
Pseudoporphyria describes a bullous photosensitivity that clinically and histologically ____________________ porphyria
cutanea tarda . However , no demonstrable porphyrin abnormalities ____________________ present . In 1964 , Zelickson
was first to describe this type of phototoxic ____________________ in patients after the use of nalidixic acid . The
skin lesions were ____________________ from those observed in patients with porphyria cutanea tarda .
Since this initial report , many other drugs have been incriminated in mediating this type of bullous
photosensitivity .
Pseudoporphyria has been ____________________ in patients with chronic renal failure treated
with and without hemodialysis and in those with excessive exposure to ultraviolet A ( UV - A ) by tanning
beds .