Coproporphyria
Overview
Coproporphyria is one of the porphyrias, a group of diseases that involves defects in heme metabolism and that results in excessive secretion of porphyrins and porphyrin precursors.
Symptoms
Symptoms include reddish-purple urine, acute neurological problems (typically episodic confusion and sensory changes), and attacks of acute abdominal/nerve pain. Around 30% suffer photosensitive skin eruptions with nail involvement; these can lead to permanent scarring.
Causes
coproporphyria is due to a combination of a genetic enzyme defect and acquired causes that become symptomatic in rare cases. In patients with coproporphyria, the function of coproporphyrinogen oxidase is only 40-60% of normal.
Treatment
While there is no cure for this condition, there are preventative measures people can take to regulate symptoms. A diet high in carbohydrates, glucose, as well as avoidance of aggravating factors (such as alcohol and drug use) can prevent attacks