title: Variable effects of beta-carotene therapy in a child with erythropoietic protoporphyria.
abstract: Erythropoietic protoporphyria (EPP) is an inborn error of heme biosynthesis with high levels of protoporphyrin in red cells and is characterized by mild to moderate photosensitivity. High-dose beta-carotene therapy has been reported to afford photoprotection in patients with EPP. We report the case of a 5-year-old Caucasian female with EPP who presented with a long-standing 3-year history of recurrent facial blisters and erythematous swelling and lesions of other sun-exposed areas of the skin. She was treated with a topical sunblocker (PreSun Ultra-SP45) but continued to show moderate to severe photosensitivity despite 3 months (March to May) of vigorous photoprotection. She was then started on increasing doses of beta-carotene (90-180 mg/day) over a period of 3 months (June to September) which resulted in a marked improvement of both facial and forearm lesions, but only modest improvement in her hand lesions.
  
  
  CONCLUSIONS
  High-dose beta-carotene appears to provide photoprotection in erythropoietic protoporphyria, resulting in improved but highly variable tolerance to sunlight.
authors.affiliation: Department of Pediatrics, University of Tennessee Medical Center, Knoxville, Tennessee, USA. ralemzad@mcw.edu
authors.forename: Ramin
authors.initials: R
authors.lastname: Alemzadeh
authors.forename: Tammy
authors.initials: T
authors.lastname: Feehan
mesh_terms: D000975:Antioxidants / Q000494:pharmacology / Q000627:therapeutic use*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D010787:Photosensitivity Disorders / Q000188:drug therapy* / Q000209:etiology; D046351:Protoporphyria, Erythropoietic / Q000150:complications / Q000188:drug therapy*; D019207:beta Carotene / Q000494:pharmacology / Q000627:therapeutic use*
chemical_list: D000975:Antioxidants; D019207:beta Carotene