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

Marcela Nowak; Dorota Mehrholz; Wioletta Barańska-Rybak; Roman Nowicki

doi:10.5114/dr.2019.89999

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

¹

,

Dorota Mehrholz

¹

,

Wioletta Barańska-Rybak

¹

,

Roman J. Nowicki

¹

Department of Dermatology, Venereology and Allergology, Medical University of Gdañsk, Poland

Dermatol Rev/Przegl Dermatol 2019, 106, 515-521

DOI: https://doi.org/10.5114/dr.2019.89999

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.

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

Marcela A. Nowak 1 , Dorota Mehrholz 1 , Wioletta Barańska-Rybak 1 , Roman J. Nowicki 1

porphyria cutanea tarda, porphyria, hormonal replacement therapy

Marcela A. Nowak

¹

,

Dorota Mehrholz

¹

,

Wioletta Barańska-Rybak

¹

,

Roman J. Nowicki

¹