Major Features of the Two Most Common Porphyrias

Porphyria

Presenting Symptoms

Exacerbating Factors

Most Important Screening Tests*

Treatment

Acute intermittent porphyria

Neurovisceral (intermittent, acute)

Alcohol ingestion

Medications (mostly cytochrome P-450 inducers)

Fasting

Infections

Organic solvents

Progesterone/progestogens

Stress/intercurrent illness

Urinary PBG

Glucose

Heme

Porphyria cutanea tarda

Blistering skin lesions (chronic)

Alcohol ingestion

Estrogens

Halogenated hydrocarbons

Hepatitis C virus

Iron

Smoking

Urinary or plasma porphyrins

Direct-acting antivirals to cure hepatitis C virus infection

Phlebotomy

* In symptomatic phase.

PBG = porphobilinogen.

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