A high skin protoporphyrin level in erythropoietic protoporphyria.
A high skin protoporphyrin level (4.36 micrograms/g wet weight) was detected in a 14-year-old girl with erythropoietic protoporphyria who died of protoporphyrin hepatopathy cirrhosis. Excess protoporphyrin was detected in the skin by our assay technique which was assumed to cause severe skin photosensitivity.
D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000032:analysis / Q000473:pathology; D008103:Liver Cirrhosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000378:metabolism*; D011166:Porphyrins / Q000032:analysis*; D011524:Protoporphyrins / Q000032:analysis*; D012867:Skin / Q000032:analysis*; D012871:Skin Diseases / Q000378:metabolism
D011166:Porphyrins; D011524:Protoporphyrins