Porphyria cutanea tarda

Question 1

Porphyria cutanea tarda most common hepatic porphyria. It is due to an inherited defect in

Answer 1

uroporphyrinogen decarboxylase

Question 2

Porphyria cutanea tarda can also be caused by ?

Answer 2

alcohol,
hepatitis C,
oestrogen

Question 3

features of Porphyria cutanea tarda

Answer 3

photosensitive rash with blistering and skin fragility on the face and dorsal aspect of hands (most common feature)

HYPERTRICHOSIS

hyperpigmentation

Question 4

Investigations

Answer 4

elevated uroporphyrinogen and pink fluorescence of urine under Wood’s lamp
serum iron ferritin level is used to guide therapy

Question 5

Management

Answer 5

chloroquine
venesection
preferred if iron ferritin is above 600 ng/ml