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Friday 24 June 2011

PORPHYRIA CUTANEA TARDA


Porphyria cutanea tarda is a chronic disturbance of the metabolism of
porphyrins, manifested by liver damage and skin lesions. The disease
affects more men than women, generally over the age of 40. Skin which
is exposed to the sun is affected, mainly the face and the backs of the
hands. Exposure to sunlight or trauma induces small blisters which lead
to erosions, crusts, atrophy, flat or depressed scars and pigmentation
changes. Milia are often present. Increased hairgrowth is also a feature.
The urine of these patients is red in colour after sun exposure due to
increased porphyrin excretion.
Management of porphyria cutanea tarda
- Avoid triggering agents, e.g. alcohol, drugs, oral contraceptives.
- Avoid trauma, avoid sun exposure.
- Phlebotomy, not to be performed in liver cirrhosis. A specialist should decide.
- Chloroquine in low doses (e.g. 150 mg weekly) for a long time.

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