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Porphyria Cutanea Tarda

Majid Ali, M.D.

 

Porphyria cutanea tarda (PCT)  is a chronic inflammatory condition of the skin and hair. It is considered to be  of uncertain origin. In my view, PCT is results from increased gut and skin fermentation caused by dysfunctional oxygen metabolism (dysox). It results from inhibition of an enzyme called UD enzyme (uroporphyrinogen decarboxylase enzyme). Most cases are associated with chronic helatitic C infection. Facial hair growth (hypertrichosis) is common. The skin abnormalities are illustrated in the photograph below (taken from The New England Journal of Medicine (2011;365:1128, september 22, 2011). The photographs are followed by text from that article.

d 2011; 365:1128September 22, 2011DOI: 10.1056/NEJMicm1100668

Article
Citing Articles (2)

A

A 51-year-old man with a history of heavy alcohol use, chronic hepatitis C virus (HCV) infection, and hepatic cirrhosis presented to his physician with an 8-month history of periorbital hair growth. On examination, healing crusts and scars were evident in sun-exposed areas. He described skin photosensitivity and intermittent painful blistering over the nape of the neck, the forearms, and the backs of the hands (Panel A). The patient's urine had pink fluorescence under a Wood's lamp, suggesting the presence of uroporphyrin (Panel B, a urine sample from the patient [indicated by an asterisk] and one from a normal control subject [N] under white light; Panel C, the same two urine samples under ultraviolet A light). A diagnosis of porphyria cutanea tarda was confirmed when marked uroporphyrinuria was shown on laboratory analysis. Porphyria cutanea tarda results from decreased activity of the uroporphyrinogen decarboxylase enzyme. Although the mechanism is unknown, the sporadic form of the disease is strongly associated with chronic HCV infection. Facial hypertrichosis is common and may serve as a diagnostic clue. Although treatment of the patient's chronic HCV infection was considered inadvisable by his hepatologist, low-dose oral hydroxychloroquine, skin photoprotection, and alcohol cessation successfully controlled the cutaneous eruptions within 6 months.

 

Chih-Chieh Chan, M.D.
Sung-Jan Lin, M.D., Ph.D.
National Taiwan University Hospital, Taipei, Taiwan 

 

Related Articles

* Metabolism

* Porphria Cutaneous Tarda

 

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