Porphyria cutanea tarda can be treated by: (LQ)
|A||High dose of systemic steroids|
|C||Low dose chloroquine|
Low dose chloroquine
1. Porphyria cutanea tarda is a group of disorders which are characterized by raised porphyrins in the plasma, urine and feces.
2. Although it is usually acquired, it can also be inherited as an autosomal dominant trait. When the disease is acquired, it is generally secondary to liver damage, most typically from alcohol abuse.
3. Alcohol and estrogens may also precipitate autosomal dominant porphyria cutanea tarda.
4. Patients typically have increased hair on the sides of the face and periorificial violaceous discolorations and blisters on the dorsum of the hands that leave scar after healing.
5. Sclerotic plaques can also develop.
6. There is marked skin fragility in sun-exposed skin areas.
7. Urine of these patients show a pink fluorescence under Wood's light.
8. Lesions similar to porphyria cutanea tarda are seen in some dialysis patients and this condition is known as "pseudo-porphyria".
9. If the disease is secondary to alcohol or estrogens, their ingestion should be stopped.
10. Patient should avoid exposure to direct sun light.
11. Venisection, the removal of 500 ml of blood every 2 weeks, results in remission.
12. Chloroquine, starting at very low doses to avoid an acute exacerbation of symptoms, is also very effective.
13. Patients with pseudo-porphyria secondary to dialysis are treated with erythropoietin in combination with venisection.