The acute porphyrias.

Public attention has been drawn to the acute porphyrias in the past few years by speculation that they affected the character of George III and the creative genius of Vincent van Gogh. During the same period, there have been important advances in the understanding of the molecular basis of the acute porphyrias and in diagnosis and the clinical management of patients and their families. Four types of porphyria are classified as acute because they produce acute neurovisceral crises (panel). Here, we outline current knowledge of the three autosomal dominant types: acute intermittent porphyria, variegate porphyria, and hereditary coproporphyria.