Variegate porphyria

The variegate porphyria (PV ) is an acute Porphyrieform with mainly neuro- visceral symptoms and occasional intolerance reactions to sunlight. Large numbers of cases occur in South Africa (founder mutation).

The PV is a congenital disorder formation of hemoglobin heme caused by the failure of one of the two genes for the enzyme protoporphyrinogen oxidase ( PPO). By the accumulation of precursors in the liver leads to sudden attacks, compared to the symptoms of acute intermittent porphyria ( AIP). The skin symptoms are easily confused with those of the hereditary coproporphyria (HCP). Factors triggering an attack are especially drugs, but also stress, alcohol consumption, hormones and hunger states.

The treatment is similar to acute intermittent porphyria. In phlebotomy and chloroquine administration, however, do not talk to patients with porphyria variegata.