Low-dose oral chloroquine in patients with porphyria cutanea tarda and low-moderate iron overload

Porfiria cutânea tardia

“…122 The duration of the remission period varies from 17 to 24 months. 122,127 Chloroquine is effective, however relapse occurs earlier than after phlebotomy. 117 There are several hypotheses to explain the mechanism of action of chloroquine: (1) it chelates iron from hepatocytes, which is later eliminated; 128 (2) it reduces ALA-synthetase activity; 129 (3) it forms a complex with uroporphyrin, which is excreted by the liver through the bile, 130 but the model utilized is not comparable to human PCT 129 , and (4) it increases the excretion of porphyrins by means of exocytosis and has a porphyrinostatic effect, inhibiting porphyrin formation.…”

“…124 Chloroquine does not worsen hepatic injury 124,126 nor causes retinopathy, when used in low doses. 127 Bullae and cutaneous fragility improve in approximately 6 months and the porphyrin excretion normalizes between six to 15 months. 117,122,124,127 It is recommended that treatment must not be interrupted until biochemical remission (urine URO < 100ìg/24h) is attained.…”

“…127 Bullae and cutaneous fragility improve in approximately 6 months and the porphyrin excretion normalizes between six to 15 months. 117,122,124,127 It is recommended that treatment must not be interrupted until biochemical remission (urine URO < 100ìg/24h) is attained. 122 The duration of the remission period varies from 17 to 24 months.…”

Hemochromatosis (HFE) Gene Mutations and Response to Chloroquine in Porphyria Cutanea Tarda

“…[22][23][24] Phlebotomy is more invasive and time-consuming and can be accompanied by hemodynamic reactions. At present, the decision on whether to use chloroquine or phlebotomy for PCT seems to be more empirical than evidence based.…”

The Cutaneous Porphyrias

“…Daily doses of chloroquine can cause a dangerous acute hepatitis, but chloroquine at the low dose of 125 mg (Malina and Chlumsky 1981 ;Ashton et al 1984 ) or 250 mg (Valls et al 1994 ;Kordac et al 1989 ) twice weekly is safe and effective. It leads to clinical remission within 6 months or so and biochemical remission after 6-15 months, at which point treatment is stopped (Malina and Chlumsky 1981 ;Ashton et al 1984 ;Valls et al 1994 ;Kordac et al 1989 ). Retinopathy does not occur with such low doses of chloroquine (Valls et al 1994 ).…”