Porphyria Cutanea Tarda (PCT)
Porphyria Cutanea Tarda (PCT)
This disease is more common in men than in women and produces cutaneous lesions on exposed portions of the body. It is frequently associated with alcoholism or hepatic disease, and toxic agents have been implicated. Genetic predisposition may play a role in some cases. Iron metabolism may be abnormal in this disorder, since most patients have hepatic siderosis.
The cutaneous manifestations begin as areas of erythema with vesicles or bullae that occur on exposed portions of the body. Crusts and scabs develop, followed by scarring. The vesicles and bullae are usually most evident in sunny weather, particularly in late summer and early autumn. In severe untreated cases, disfiguring changes can occur in the ears, nose and fingers.
Liver disease is present in many patients with PCT. Histologically, the most frequent findings are siderosis and evidence of recurring liver damage. Some patients have frank cirrhosis. Liver fluorescene may be demonstrable. Chloroquine produces a reaction in this disease that can include fever, headache, abdominal pain, vomiting and red urine.
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