Porphyria cutanea tarda Flashcards
1
Q
Porphyria cutanea tarda most common hepatic porphyria. It is due to an inherited defect in
A
uroporphyrinogen decarboxylase
2
Q
Porphyria cutanea tarda can also be caused by ?
A
alcohol,
hepatitis C,
oestrogen
3
Q
features of Porphyria cutanea tarda
A
photosensitive rash with blistering and skin fragility on the face and dorsal aspect of hands (most common feature)
HYPERTRICHOSIS
hyperpigmentation
4
Q
Investigations
A
elevated uroporphyrinogen and pink fluorescence of urine under Wood’s lamp
serum iron ferritin level is used to guide therapy
5
Q
Management
A
chloroquine
venesection
preferred if iron ferritin is above 600 ng/ml
