Write about non-acute porphyrias here.

• Normal �-ALA and porphobilinogen

• Raised porphobilinogen deaminase

Porphyria Cutanea Tarda

(Cutaneous hepatic porphyria)

• Genetic predisposition

• Bullous eruption on exposure to sunlight, heals without scarring

• EtOH most common aetiological agent

• Evidence of biochem or clinical liver disease may also be present.

• Some association with benign or malignant tumours of the liver

• Increased urinary uroporphyrin

• Remission may be induced using venesection

Erythropoietic Porphyria:

Congenital Porphyria

• Extremely rare

• Autosomal recessive

• Extreme sensitivity to sunlight and disfiguring scars

Erythropoietic protoporphyria

• More common than CP above.

• Autosomal dominant

• Irritation and burning pain on exposure to sunlight

• Oral β-carotene provides effective protection against solar sensitivity through an unknown mechanism.