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Porphyria Cutanea Tarda Brief

 

 

Definition

Porphyria cutanea tarda is the most common type of porphyria.

Prophyria cutanea tarda (PCT) is due to a defective enzyme in the liver (uroporphyrinogen decarboxylase) involved in synthesis of the red pigment in blood cells (haem).

The results is an increase in porphyrins in the skin, resulting in photosensitivity i.e. the skin is damaged by light.

Affected individuals develop fragile skin, sores (erosions), blisters (vesicles and bullae), and tiny cysts (milia) on the sun-exposed areas i.e. the back of the hands and the forearms. They may notice that they sunburn easily. Some people develop mottled brown patches around the eyes and increased facial hair. Occasionally the skin becomes hardened (sclerodermoid) on the neck, face or chest. There may be small areas of permanent baldness (alopecia) or ulcers.

 

 

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