Liver Iron Overload and Desferrioxamine Treatment of Porphyria cutanea tarda

Rocchi, Emilio; Cassanelli, M.; Borghi, A.; Paolillo, F; Pradelli, M.; Pellizzardi, S.; Vezzosi, A.; Gallo, E.; Contri, M. Baccarani; Ventura, Ezio

doi:10.1159/000247732

Cited by 30 publications

(12 citation statements)

References 11 publications

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“…However, there are no controlled studies on the use of these medications in PCT. Additionally, the use of iron chelators is associated with potentially severe side effects and, in small studies, iron chelation has been found to be less efficient than phlebotomy …”

Section: Porphyria Cutanea Tardamentioning

confidence: 99%

“…Additionally, the use of iron chelators is associated with potentially severe side effects and, in small studies, iron chelation has been found to be less efficient than phlebotomy. 63,77 Traditionally, iron chelation was performed with desferrioxamine. As desferrioxamine has a short half-life and cannot be absorbed enterally, it is administered either IV overnight or via subcutaneous injection twice daily.…”

Section: Iron Chelationmentioning

confidence: 99%

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Cutaneous porphyrias part II: treatment strategies

et al. 2013

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The porphyrias are diverse in pathophysiology, clinical presentation, severity, and prognosis, presenting a diagnostic and therapeutic challenge. Although not easily curable, the dermatological manifestations of these diseases, photosensitivity and associated cutaneous pathology, can be effectively prevented and managed. Sun avoidance is essential, and patient education regarding the irreversibility of photocutaneous damage is a necessary corollary. Beyond preventative measures, the care of fragile, vulnerable skin, and pain management, each of the porphyrias has a limited number of unique additional therapeutic options. Many of the treatments have been published only in small case series or anecdotal reports and do not have well-understood nor proven mechanisms of action. This article presents a comprehensive review of available therapeutic options and long-term management recommendations for the cutaneous porphyrias.

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Section: Porphyria Cutanea Tardamentioning

confidence: 99%

Section: Iron Chelationmentioning

confidence: 99%

Cutaneous porphyrias part II: treatment strategies

et al. 2013

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“…However, cost-effectiveness is lower than in simple phlebotomy. When phlebotomy is impossible intravenous application of desferroxamine has been suggested as an alternative [35], but its efficacy is not proven.…”

Section: Iron Depletion Therapymentioning

confidence: 99%

Therapy of porphyria cutanea tarda

Köstler¹,

Wollina²

2005

Expert Opinion on Pharmacotherapy

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Porphyria cutanea tarda (PCT) is the most common type of porphyria. There is an association of PCT with haemochromatosis, diabetes mellitus and hepatitis C infection. The basis of treatment of PCT consists of three elements: avoidance of triggering factors, iron depletion and porphyrin elimination. Alcohol and certain systemic medical drugs, such as oestrogens (or tuberculostatics), should be considered as triggering factors, and as far as possible, avoided. Other triggering factors, such as chronic haemodialysis in renal insufficiency, need a different approach. The hallmark in iron depletion is phlebotomy. Porphyrin elimination is achieved using low-dose chloroquin therapy. The treatment is safe and effective but has its limits in cases with haemochromatosis (HFE) gene mutations. Here iron depletion needs additional phlebotomy. In patients with chronic haemodialysis-associated PCT, chloroquine is ineffective. Erythropoietin, desferroxamine and small-volume phlebotomy have been employed to control the disease. Childhood PCT is very rare. No controlled studies are available, but published experience suggests that body weight-adapted chloroquine therapy or small volume phlebotomy might be useful.

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“…The subcutaneous and intravenous administration of desferrioxamine is another possibility to reduce the iron levels and improve signs of disease [81].…”

Section: Reduction Of the Iron Levelmentioning

confidence: 99%

Porphyria cutanea tarda

Fritsch

Lang

Schmiedeberg

et al. 1998

Skin Pharmacol Physiol

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Porphyria cutanea tarda (PCT) is the most frequent form of porphyria. The underlying enzymatic defect in PCT is a reduced activity of the enzyme uroporphyrinogen decarboxylase (Uro-D). Four different types of Uro-D disturbances are known. Pseudoporphyrias such as porphyria cutanea uraemica or drug-induced PCT-like skin symptoms are distinguished from PCT. Porphyrinogens such as estrogens or alcohol, or other inducers of P450 isoenzymes provoke PCT. Polymorphisms of P450 isoenzymes, iron overload and infection with hepatitis C virus play an important role in the etiopathogenesis of disease manifestation. Dominant clinical symptoms are bullae, increased cutaneous vulnerability, hypertrichosis and elastosis. Biochemically, total porphyrin levels in urine are increased with a predominance of uroporphyrin and heptacarboxylic porphyrin. Isocoproporphyrin is demonstrable in feces. Best therapeutic strategies are the oral administration of chloroquine 125 mg twice a week and repetitive bloodlettings or the combination of both.

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Liver Iron Overload and Desferrioxamine Treatment of Porphyria cutanea tarda

Cited by 30 publications

References 11 publications

Cutaneous porphyrias part II: treatment strategies

Cutaneous porphyrias part II: treatment strategies

Therapy of porphyria cutanea tarda

Porphyria cutanea tarda

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