Porphyria Cutanea Tarda Flashcards
1
Q
What is Porphyria cutanea tarda?
A
It is either a genetic or aquired disorder that results low levels of the enzyme responsible for the fifth step in heme production.
It is named because it often presents with skin manifestations.
2
Q
How is it aquired?
A
Hepatocyte damange (alcohol, hepatitis, oestrogens.)
3
Q
What are the features of porphyria cutanea tarda?
A
- Classically presents with photosensitive rash with blistering and skin fragility on the face and dorsal aspect of hands (most common feature)
- Hypertrichosis
- Hyperpigmentation
4
Q
How is it investigated?
A
- Urine - elevated uroporphyrinogen
- Pink flourescence of urine under a Wood’s lamp
5
Q
How is porphyria cutanea tarda managed?
A
- Chloroquine
- Venesection
6
Q
A
