Hereditary coproporphyria

Classification according to ICD-10
E80	Disorders of the porphyrin and bilirubin metabolism
E80.2	Hereditary coproporphyria
ICD-10 online (WHO version 2019)

The Hereditary coproporphyria ( HCP ) is an acute, hepatic (liver concerned) metabolic disorder is selected from the group of porphyria . These diseases affect the synthesis of porphyrins such as heme , the red blood pigment. HCP mainly shows symptoms in the nervous system and internal organs ( neurovisceral ), more rarely also sun intolerance.

HCP is a congenital disorder of the formation of the red blood pigment hemoglobin , caused by the partial failure of the enzyme coproporphyrinogen oxidase ( CPO , 6th enzyme in the heme formation chain). The reduced activity of the enzyme leads to the accumulation of precursors ( coproporphyrins ) in the liver and in the feces. HCP is inherited as an autosomal dominant trait. Symptoms, triggering factors and treatment are the same as for acute intermittent porphyria (AIP) , with the difference that in some cases sunlight is not tolerated.

Web links

• Hereditary coproporphyria.  In: Online Mendelian Inheritance in Man . (English).

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