JLE

European Journal of Dermatology

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Journal Club Volume 30, numéro 6, November-December 2020

Auteurs
1 Dermatology Department, Edouard Herriot Hospital, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, LyonFrance
2 Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, WürzburgGermany
3 Department of Dermatology, Centro Hospitalar Universitário do Porto, PortoPortugal
4 Department of Dermatology, Hospital General Universitario Gregorio Marañón, MadridSpain
5 Department of Dermatology, Oxford University Hospitals NHS Foundation Trust, Churchill Hospital, OxfordUK

Porphyria cutanea tarda (PCT) is a rare metabolic disease. It is due to a reduced activity of uroporphyrinogen decarboxylase (UROD) caused by several factors: genetic mutations (in HFE or UROD), alcohol abuse, smoking, or HCV or HIV infection [1]. This deficiency results in an accumulation of porphyrins in the liver and skin, causing cutaneous symptoms such as photosensitivity, blisters, hyperpigmentation and hypertrichosis on sun-exposed skin. PCT is the most frequent human porphyria and its prognosis [...]