Variable effects of beta-carotene therapy in a child with erythropoietic protoporphyria.

Abstract

UNLABELLED:

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.

CONCLUSION:

High-dose beta-carotene appears to provide photoprotection in erythropoietic protoporphyria, resulting in improved but highly variable tolerance to sunlight.

• Authors: Ramin Alemzadeh, Tammy Feehan
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 163 Issue 9 Pg. 547-9 (Sep 2004) ISSN: 0340-6199 [Print] Germany
• PMID: 15241683 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antioxidants
• beta Carotene

Topics

• Antioxidants (pharmacology, therapeutic use)
• Child, Preschool
• Female
• Humans
• Photosensitivity Disorders (drug therapy, etiology)
• Protoporphyria, Erythropoietic (complications, drug therapy)
• beta Carotene (pharmacology, therapeutic use)