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Congenital Erythropoietic Porphyria

MICHELINE M. MATHEWS-ROTH, MD
Am J Dis Child. 1977;131(3):366. doi:10.1001/archpedi.1977.02120160120022.
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Sir.—The picture of the month in the June 1975 issue of the Journal concerned itself with a case of congenital erythropoietic porphyria. The discussion suggested the use of a topical light-screening cream for the management of the photosensitivity associated with this disease. Another therapeutic agent for the management of the photosensitivity—the use of orally administered beta-carotene (Solatene)-may be more effective. Although beta-carotene is used most often in the therapy of erythropoietic protoporphyria,1 there have appeared two reports of the successful use of oral beta-carotene in congenital erythropoietic porphyria, resulting in significant improvement in the patients' photosensitivity.2:i

We have administered beta-carotene to two additional patients with congenital erythropoietic porphyria. One patient, a 9-year-old boy (patient of T. R. K.), had a significant reduction in number and severity of his skin lesions and has been able to increase his sun exposure while receiving therapy (180 mg/day). Beta-carotene has not altered

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