[Does paraneoplastic porphyria cutanea tarda exist?].

Area of Science:

• Hepatology
• Dermatology
• Oncology

Context:

• Porphyria cutanea tarda (PCT) is a metabolic disorder affecting the liver and skin.
• The relationship between PCT and cancer, particularly paraneoplastic dermatoses, requires clarification.
• Distinguishing PCT from other conditions with similar presentations is crucial for accurate diagnosis and management.

Purpose:

• To investigate the association between porphyria cutanea tarda and various types of tumors.
• To determine if PCT is a paraneoplastic dermatosis or if it correlates with specific cancers.
• To evaluate the potential role of chloroquine phosphate in managing PCT and its associated risks.

Summary:

• Biochemical diagnoses in 134 patients confirmed porphyria cutanea tarda (PCT) is generally not a paraneoplastic dermatosis.
• While rare, a hepatoma producing porphyrins could be indicated by irregular urinary porphyrin excretion with hepatic porphyria skin changes.
• Patients with long-standing PCT and liver cirrhosis exhibit a higher incidence of hepatocellular carcinoma compared to cirrhotics without PCT.
• Chloroquine phosphate therapy may potentially prevent the progression of liver disease in PCT patients towards hepatocellular carcinoma.

Impact:

• Clarifies that PCT is not typically a paraneoplastic syndrome, reducing diagnostic confusion.
• Highlights a potential increased risk of hepatocellular carcinoma in long-term PCT patients with liver cirrhosis.
• Suggests chloroquine phosphate as a potential therapeutic strategy to mitigate cancer risk in PCT patients.