Coproporphyria, Hereditary

**Definition:** An autosomal dominant porphyria that is due to a deficiency of COPROPORPHYRINOGEN OXIDASE in the LIVER, the sixth enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include both neurological symptoms and cutaneous lesions. Patients excrete increased levels of porphyrin precursors, 5-AMINOLEVULINATE and COPROPORPHYRINS.

**Tree numbers:** - C06.552.830.074 - C16.320.850.742.074 - C17.800.827.742.074 - C18.452.811.400.074

**Synonyms:** - Coproporphyrinogen Oxidase Deficiency - Hereditary Coproporphyria