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.
