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Recurrent painful hand crisis in a four-year-old girl, revealing an erythropoietic protoporphyria


European Journal of Dermatology. Volume 8, Number 7, 515-6, October - November 1998, Cas cliniques

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Author(s) : Anne-Laure LABROUSSE, Véronique SALMON-EHR, Catherine ESCHARD, Bernard KALIS, Fabienne LEONARD, Philippe BERNARD

Summary : We report the case of a 4-year-old girl, who had been suffering for 2 years from a recurrent, painful crisis affecting both hands, following sun exposure. There were no obvious cutaneous lesions, which initially caused us to consider a diagnosis of a psychiatric disorder. However, the diagnosis of erythropoietic protoporphyria was then established by the demonstration of elevated levels of free protoporphyrin in erythrocytes. The present case illustrates the effectiveness of beta-carotene associated with canthaxanthin in erythropoietic protoporphyria.

Keywords : erythropoietic protoporphyria, photosensitivity.

 

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