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Dermatology > Porphyrias > Flashcards

Flashcards in Porphyrias Deck (9):
1

What is photo dermatology

Use of light radiation for diagnostic and therapeutic cases
E.g. UV light can help treat skin conditions

2

What is the Fitzpatrick scale

A scale of skin types by how photosensitive they are
Class 1 is most likely to burn etc

3

What is the cause of many porphyrias

A defect in one of the enzymes in the haem pathway
Leads to a build up of porphyrin
Different enzyme defects cause different types

4

What are the 4 main groups of porphyrias

Photo toxic skin porphyria - erythropoietic
Blistering and skin fragility porphyria
Acute attack
Severe congenital

5

How do you diagnose porphyria cutanea tarda

Look for clinical signs - blisters, fragile skin (often on backs of hands)
Take a urine sample and shine a woods lamp through it - the excess porphyrins cause it to go a different colour

6

What are some non-skin affects of erythropoietic protoporphyria

Mild anaemia
Gallstones
Rarely liver failure

7

What investigations can be done for EPP

Quantative RBC porphyrins
Transaminases
Flurocytes

8

What can be done to manage EPP

Genetic counselling and advice on condition
6 months of LFT and RBC porphyrin checks
Protection from visible light - long, dark clothing etc
Phototherapy - exposure to build tolerance
Anti-oxidants

9

List some protective measures from UV light

Behavioural - avoiding midday
Clothing
Environmental - staying in shade, having blinds drawn
Sunscreen