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SOME CLINICAL AND FUNDAMENTAL ASPECTS OF ERYTHROPOIETIC PROTOPORPHYRIA

D. Suurmond, J. Van Steveninck, L.N. Went

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Abstract

Summary— The cutaneous manifestations observed in 17 patients with erythropoietic protoporphyria are briefly discussed. A fluorocyte concentration test has proved to be useful for screening protoporphyria families. An accelerated in vitro photohaemolysis could be demonstrated not only in blood from protoporphyria patients, but also in normal blood to which a protoporphyrin ester has been added. A number of reducing agents appeared to be more or less potent inhibitors of the protoporphyrin‐induced photohaemolysis.

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Summary— The cutaneous manifestations observed in 17 patients with erythropoietic protoporphyria are briefly discussed. A fluorocyte concentration test has proved to be useful for screening protoporphyria families. An accelerated in vitro photohaemolysis could be demonstrated not only in blood from protoporphyria patients, but also in normal blood to which a protoporphyrin ester has been added. A number of reducing agents appeared to be more or less potent inhibitors of the protoporphyrin‐induced photohaemolysis.

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Available abstract

Summary— The cutaneous manifestations observed in 17 patients with erythropoietic protoporphyria are briefly discussed. A fluorocyte concentration test has proved to be useful for screening protoporphyria families. An accelerated in vitro photohaemolysis could be demonstrated not only in blood from protoporphyria patients, but also in normal blood to which a protoporphyrin ester has been added. A number of reducing agents appeared to be more or less potent inhibitors of the protoporphyrin‐induced photohaemolysis.

Key concepts: Erythropoietic protoporphyria, Protoporphyrin, Medicine, Dermatology, Ferrochelatase, Porphyria, Heme, Chemistry

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