Chronic hepatic porphyria

Orpha code: 95161OMIM code:

Definition

Chronic hepatic porphyrias represent a sub-group of porphyrias (see this term). They are characterized by bullous photodermatitis caused by a deficiency of uroporphyrinogen decarboxylase (URO-D; the fifth enzyme in the heme biosynthesis pathway). Chronic hepatic porphyria encompasses two diseases: porphyria cutanea tarda and hepatoerythropoietic porphyria (extremely rare) (see these terms).

Disease data
Classification

Clinical group

ORPHA code
95161
OMIM code
-
ICD10 code
E80.2
ICD11 code
-

No additional description.

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