Variegate porphyria: Past, present and future

Variegate porphyria, one of the acute hepatic porphyrias, is characterized by a partial reduction in protoporphyrinogen oxidase, the seventh enzyme of the heme biosynthetic pathway. For a long time, this disease has caused co nfusion among the porphyrias because it presents with clinical symptoms and biochemical findings that can be similar to those found in other types of porphyrias, Here, we provide an overview of historical, clinical, biochemic al, genetical, and other aspects of variegate porphyria that might be helpf ul in providing more insight into this rare disorder.