How is cutaneous porphyria treated?

Updated: Aug 31, 2020
  • Author: Brenda Chiang, DO; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Answer

Cutaneous porphyrias

  • Avoidance of sunlight is the key in treating cutaneous porphyrias. [84]
  • The use of beta-carotene has shown some benefit for cutaneous porphyrias.

  • Porphyria cutanea tarda can be effectively treated by phlebotomy. [85]  Phlebotomy has also proved effective in treatment of congenital erythropoietic porphyria, [86]

  • High-dose chloroquine therapy for porphyria cutanea tarda is rarely used now because of its hepatic effects. [87] Low-dose chloroquine appears at least as effective as phlebotomy. [88]  A meta-anaysis found that relapse rates in the year following treatment were 20% with phlebotomy and 35-36% with chloroquine. [89]

  • Iron chelation therapy (eg, with deferasirox, deferiprone, or desferrioxamine) may be considered for patients with porphyria cutanea tarda if phlebotomy is contraindicated. In a pilot study of 10 patients with porphyria cutanea tarda, iron chelation with deferasirox reduced new blisters and ferritin levels in most patients. [90]

  • Afamelanotide, an α-melanocyte–stimulating hormone analogue, has been shown to permit increased duration of sun exposure without pain and improve quality of life in patients with erythropoietic protoporphyria. [91, 92] It is administered in a subcutaneous implant containing a 60-day supply of the medication. The US Food and Drug Administration approved afamelanotide (Scenesse) in October 2019. [93]


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