25-11-2013
Porphyrias are a group of diseases associated with a disorder of heme synthesis. Late cutaneous porphyria is the most common. It occurs in familial and sporadic forms. It usually affects adults who develop hepatitis. It is possible to carry the genetic defect without symptoms.
The most common symptom is excessive skin sensitivity to sunlight. Often exposure to factors such as alcohol, hepatitis virus, drugs or other hepatotoxic substances is necessary for symptoms to occur. Patients develop blisters, which may progress to erosions and leave scars.
Post-inflammatory hyperpigmentation and scleroderma-like lesions are common. The skin lesions are localised on exposed parts of the body. The hands, face and forearms are the most common. Increased skin sensitivity is noted in patients. In addition to the skin symptoms, the condition may manifest as dark urine staining.
Cutaneous porphyria responds well to treatment with antimalarial drugs. The initial exacerbation of skin lesions is associated with the secretion of larger amounts of porphyrins.
The effectiveness of treatment depends on the removal of agents that are harmful to the liver. It is important to use protective measures against UV radiation.
