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

Ramin Alemzadeh, Tammy Feehan

Department of Pediatrics, University of Tennessee Medical Center, Knoxville, Tennessee, USA. ralemzad@mcw.edu

European journal of pediatrics 2004 Sep

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

Citation

Ramin Alemzadeh, Tammy Feehan. Variable effects of beta-carotene therapy in a child with erythropoietic protoporphyria. European journal of pediatrics. 2004 Sep;163(9):547-9