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Review
. 2016 Mar;67(3):207-10.
doi: 10.1007/s00105-015-3744-4.

[Porphyria cutanea tara]

[Article in German]
H F Merk  1   2
Affiliations
Review

[Porphyria cutanea tara]

[Article in German]
H F Merk. Hautarzt. 2016 Mar.

Abstract

Porphyria cutanea tara (PCT) has a prevelance of about 40 new diagnoses per 1 million people per year and is the most frequently occurring type of porphyria worldwide. Inhibition of the uroporphyrinogen decarboxylase (UROD) is the main cause of the disease, which can be the result of a heterozygous or homozygous mutation of the UROD gene; however, xenobiotics or other diseases may play an important role for the precipitation of the disease. Risk factors include alcohol, estrogen, iron overload, and hemochromatosis, hepatitis C or poisoning, e.g., with polyhalogenated aromatic compounds such as hexachlorobenzene. Signs and symptoms are blisters, skin fragility, erosions hyperpigmentation, sclerodermoid plaques. Therapy includes sun protection, prevention of risk factors, phlebotomy, and chloroquine.

Keywords: Aryl-hydrocarbon-receptor; Chloroquine; Hexachlorobenzene; Sunscreening agents; Uroporphyrinogen decarboxylase.

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