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September 20, 1965

Erythropoietic ProtoporphyriaI. Clinical and Laboratory Features in Seven New Cases

Author Affiliations

From the Department of Medicine, divisions of dermatology (Drs. Peterka, Fusaro, and Jaffe) and internal medicine (Drs. Runge and Watson), University of Minnesota Hospital, Minneapolis.

JAMA. 1965;193(12):1036-1042. doi:10.1001/jama.1965.03090120044011

For many years attempts have been made to associate "polymorphic light eruptions," such as urticaria solaris, eczema solare, and purpura solaris with abnormalities of porphyrin metabolism. Hydroa aestivale or vesiculobullous photocutaneous lesions occur in congenital erythropoietic porphyria (Morbus Günther) and with cutaneous forms of hepatic porphyria, in all of which the urinary uroporphyrin is invariably increased and characteristic, while in the polymorphous eruptions just alluded to, the urinary porphyrin is normal. In 1953, Kosenow and Treibs1 reported the case of a 12-year-old male with atypical hydroa aestivale without excessive urinary porphyrin but with elevated erythrocyte protoporphyrin (EP) and fecal protoporphyrin (FP). Although this probably represents the first recorded example of what has come to be designated as erythropoietic protoporphyria,2 interpretation is somewhat complicated by the presence in the patient's serum of a porphyrin metal complex, the absorption spectrum of which corresponded with that of uroporphyrin or coproporphyrin rather

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