Conditions / Genetic

erythropoietic protoporphyria

info · Genetic · ICD-10: E80.0

An acute porphyria characterized by a deficiency in the enzyme ferrochelatase, leading to abnormally high levels of protoporphyrin in the tissue.

Signs and symptoms

  • Hepatic failure
  • Hypertriglyceridemia
  • Edema
  • Low tissue ferrochelatase activity
  • Hemolytic anemia
  • Cholelithiasis
  • Eczematoid dermatitis
  • Pruritus
  • Erythema
  • Increased erythrocyte protoporphyrin concentration

Medications that may treat it

afamelanotide methoxsalen

Also known as: EPP; Protoporphyria