Variegate Porphyria (VP)
This form of hepatic Porphyria is most common in the white South African population. It is less common elsewhere. It is an autosomal dominant disorder and may produce acute attacks (as in AIP) as well as skin photosensitivity. The deficient enzyme is protoporphyrinogen oxidase. The diagnosis is made by finding excess coproporphyrin in urine and both coproporphyrin and protoporphyrin in feces. The most sensitive screening test for VP is probably a plasma porphyrin assay. In patients with skin manifestations, it is important to distinguish VP or HCP from PCT, because treatment by phlebotomy or low-dose chloroquine is not successful in VP and HCP. Acute attacks are managed and may be prevented as in AIP.
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