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December 1964

The Porphyrias: A Story of Inheritance and Environment

Author Affiliations

Mayo Clinic Rochester, Minn 55901

Arch Dermatol. 1964;90(6):629. doi:10.1001/archderm.1964.01600060095018

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This small volume is not intended to be a text on the subject of porphyria, although the various forms in which the disease becomes manifest, either by inheritance or as a result of toxic factors, or both, are clearly set forth.

The main theme deals with what has been called porphyria variegata, a mixed type of porphyria occurring exclusively among the Boers of South Africa, having been passed down by dominant inheritance through some 13 generations and stemming from an original family who settled at the Cape in the latter part of the 17th century. Porphyria variegata appears in two forms: (1) In women who inherit the trait, the disease may remain latent for life in spite of multiple pregnancies; or, and especially under the precipitating effect of the administration of barbiturates or sulfonamides, all the signs of acute intermittent porphyria may occur. (2) In men who inherit the trait,

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