Therapeutic strategies:
(1) skin protection
(2) avoidance of precipitating factors
(3) reducing body iron stores
(4) increasing excretion of porphyrins into the urine
Skin protection:
(1) covering up with long sleeves and a hat
(2) minimizing exposure to ultraviolet lights, especially at peak sunlight periods
(3) applying suntan lotions
Avoidance of precipitating factors:
(1) avoiding or minimizing the use of alcohol
(2) avoiding chlorinated hydrocarbons
(3) if female, avoiding estrogens and oral contraceptives
If iron overload is present, then the body iron stores can be reduced by repeated phlebotomy with removal of 450-500 mL whole blood until the patient is anemic (hemoglobin 10 to 11 g/dL). A less effective alternative is therapy with the iron chelator DFO.
Therapy with low dose chloroquine or hydroxychloroquine can increase porphyrin excretion, which reduces the levels in the liver. Chloroquine may also reduce uroporphyrin synthesis. A patient starting therapy may show a transient worsening of symptoms as porphyrins are mobilized.
A patient with renal failure may not be able to excrete the porphyrins in the urine and may have chronic anemia. Treatment with erythropoietin may increase red cell production enough to allow repeated phlebotomy. High flux hemodialysis may be more effective in removing porphyrins than regular hemodialysis if plasma levels are high.
