Coproporphyria

Corpropohyria is an autosomal dominant disease that has the same precipitating factors as acute intermittent porphyria. The disease can remain latent indefinitely until an acute attack is precipitated. In acute attacks both neurologic and cutaneous manifestations occur.

Laboratory Findings.The disorder is due to decreased coproporphyrinogen oxidase activity which causes increased excretion of fecal coproporphyrin. Urine coproporphyrin may or may not be increased and if increased without any other urine findings is not specific for porphyria since it can also be found in malignant, hepatic, hematologic, and toxic disorders. In acute attacks urinary ALA and porpphobilinogen can be increased, but may return to normal when the patient is without symptoms.

Treatment. The management is the same as for acute intermittent and variegate porphyria.