Erythropoietic protoporphyria.

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Abstract
The terminal stages of erythropoietic protoporphyria were recorded. The observations were related to the site of the fundamental lesion and the nature of the biochemical defect. The possibly ominous prognosis in this usually mild condition was emphasized. Apart from congenital porphyria, the porphyrias do not usually confer severe cutaneous lesions. These diseases present to dermatologists because of moderate photosensitivity and are not usually regarded as a risk to life. Dangerous CNS involvement may occur in acute intermittent, variegate and hereditary coproporphyrias, while in acquired symptomatic porphyria, severe underlying liver dysfunction may occur. Probably the most common familial photosensitizing porphyria is erythropoietic protoporphyria. Recently some deaths from severe liver involvement were reported in this disease.