Porphyria Cutanea Tarda

 Porphyria cutanea tarda :

It results from underactivity of the enzyme uroporphyrinogen decarboxylase, which leads to accumulation of porphyrins in the liver. 

Liver disease is common. 

About 35% of people develop cirrhosis and 7 to 24% develop liver cancer.

Symptoms of Porphyria:

The acute (hepatic) porphyrias are characterized by episodes of abdominal pain (the most common symptom), constipation and other gastrointestinal symptoms.

Photosensitivity leading to pain immediately upon exposure to sunlight (e.g. EPP) or blistering and fragility of the skin in areas exposed to sunlight is seen in Porphyria cutanea tarda. 

How is PCT diagnosed?

 Diagnosisof (PCT) relies on the identification of characteristic symptoms, a comprehensive patient history, and a thorough clinical evaluation involving specialized tests.

Diagnosis is established by finding substantial increases in porphyrins in urine or plasma and excluding other blistering cutaneous porphyrias.

What is the drug of choice for porphyria cutanea tarda? :

Porphyria cutanea tarda may be treated by simply avoiding the precipitating factors.

 However, phlebotomy and hydroxychloroquine are both effective in achieving biochemical and clinical remission.