eISSN: 2084-9893
ISSN: 0033-2526
Dermatology Review/Przegląd Dermatologiczny
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5/2019
vol. 106
 
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abstract:
Case report

Early diagnosis of porphyria cutanea tarda as a key to avoiding scarring – a mild form of the disease

Marcela A. Nowak
,
Dorota Mehrholz
,
Wioletta Barańska-Rybak
,
Roman J. Nowicki

Dermatol Rev/Przegl Dermatol 2019, 106, 515-521
Online publish date: 2019/12/06
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Porphyria cutanea tarda belongs to the group of porphyrias. Porphyria is a heme disorder caused by an acquired or congenital defect of hepatic enzymes. This consequently leads to the accumulation of porphyrins in various organs. In porphyria cutanea tarda, there is a deficiency of the enzyme uroporphyrinogen decarboxylase. The most frequent factor that leads to damage of this enzyme is drug- or alcohol-induced liver insufficiency. In porphyria cutanea tarda typical clinical manifestations include blisters, erosions, crusts, and milia, that arise predominantly in sun-exposed areas. Furthermore, the skin becomes atrophic, susceptible to injuries and scarring. In our patient, the skin chances were induced by estrogen treatment of endometriosis in combination with UV-exposure. In this case the diagnosisi was established early and significant clinical consequences could be avoided.
keywords:

porphyria cutanea tarda, porphyria, hormonal replacement therapy

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