Porphyria

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Porphyria cutanea tarda on the hands

Porphyria

Alternative Names:

Acute intermittent porphyria; Hereditary coproporphyria; Congenital erythropoietic porphyria; Erythropoietic protoporphyria
Treatment:

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During an acute attack, these treatments can be expected.

Maintenance of electrolyte balance
Monitoring of blood gasses
Increase of carbohydrate intake (may be IV glucose solutions)
Pain medication as needed
Sedation for restlessness and agitation
Propranolol for hypertension
Intravenous hematin

Long-term management:

Avoid all alcohol
Avoid drugs that may precipitate an attack
Eat high carbohydrate diet
Avoid sunlight
Use sunscreen (if not contraindicated)
Avoid skin trauma
Beta-carotene
Consider splenectomy

Expectations (prognosis):

The porphyrias are life-long intermittent illnesses, but with good long-term management, the affected person can expect long problem-free periods.

Complications:

Progressive muscle paralysis
Respiratory failure
Gallstones
Disfigurement
Coma

Calling your health care provider:

Call your health care provider if you have any of the above symptoms suggestive of a porphyria. If you have already been diagnosed with a porphyria, call your health care provider at the first signs of an acute attack.