Porphyria
Most Common Type: Porphyria Cutanea Tarda
Basic enzyme deficiency: uroporphyrinogen decarboxylase
Maximum Phototoxicity.
Psychiatric manifestation: Acute Intermittent Porphyria
Phototoxicity NOT seen: Acute Intermittent Porphyria
Erythropoietic protoporphyria: Ferrochelatase deficiency
Congenital Erythropoietic porphyria: (Only porphyria with A.R)Uroporphyrinogen III synthase
Pseudoporphyria: A bullous photosensitivity that clinically and histologically mimics PCT.chronic renal failure treated with hemodialysis,exposure to ultraviolet A (UV-A), Drugs.
Variegate porphyria: protoporphyrinogen oxidase.
cutaneous photosensitivity, systemic symptoms arising from neurologic dysfunction, or both.
Acute Intermittent Porphyria, Varigate Porphyria and Congenital erythropoietic porphyria produce increase in urine prophobilinogen.
Wednesday, 7 January 2009
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