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

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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 affford 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.

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Abbreviations

EPP :

erythropoietic protoporphyria

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Author notes

  1. Ramin Alemzadeh

    Present address: Medical College of Wisconsin, 8701 Watertown Plank Road, 53226, Milwaukee, WI , USA

Authors and Affiliations

  1. Department of Pediatrics, University of Tennessee Medical Center, Knoxville, Tennessee, USA

    Ramin Alemzadeh & Tammy Feehan

Authors
  1. Ramin Alemzadeh
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  2. Tammy Feehan
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Correspondence to Ramin Alemzadeh.

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Alemzadeh, R., Feehan, T. Variable effects of beta-carotene therapy in a child with erythropoietic protoporphyria. Eur J Pediatr 163, 547–549 (2004). https://doi.org/10.1007/s00431-004-1453-6

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  • DOI: https://doi.org/10.1007/s00431-004-1453-6

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