What is the pathophysiology of variegate porphyria?

Updated: Aug 31, 2020
  • Author: Brenda Chiang, DO; Chief Editor: Emmanuel C Besa, MD  more...
  • Print


Variegate porphyria is an autosomal dominant inherited trait that results in decreased activity of protoporphyrinogen oxidase. It is characterized clinically by photosensitive skin disease and a propensity to acute neurovisceral crises. It presents with chronic blistering skin lesions that are less prevelant in colder regions and dark-skinned individuals. [20] Variegate porphyria is found worldwide but has an exceptionally high frequency in South Africa. [21]

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!