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Hereditary coproporphyria

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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Hereditary coproporphyria is an autosomal dominant deficiency of coproporphyrinogen oxidase (chromosome 9) in white blood cells, liver and other cells. Two-thirds of patients are latent. It is very rare and may present at any age. It is slightly more common in females.

The clinical features are predominantly visceral and neurological. Acute attacks resemble acute intermittent porphyria and variegate porphyria, and may be precipitated by the same agents. The patient may present with chronic "nervousness" and other psychiatric complaints, with or without abdominal pain. Photosensitivity occurs infrequently.

Investigation reveals increased levels of faecal and urinary coproporphyrinogen.

Treatment is by avoidance of precipitating agents.

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