Photodermatology and the porphyrias

Question 1

What are the 2 main therapeutic treatments for photodermatology

Answer 1

PUVA

UVB

Question 2

UVA causes sunburn reactions. True or False

Answer 2

False - it is UVB

Question 3

What are the 4 main groups of porphyrias

Answer 3

Phytotoxic skin porphyrias
Blistering and fragility skin porphyrias
Acute attack porphyries
Severe congenital prophyrias

Question 4

What are the common skin features of skin porphyria

Answer 4

Blistering and fragility 
hyperpigmentation
hypertrichosis
solar urticaria
morphoea

Question 5

What is the most common type of skin porphyria in scotland

Answer 5

Porphyria cutanea tarda (PCT)

Question 6

What investigations should be done for porphyria?

Answer 6

Urine tested using a Woods lamp

Spectrophotometer

Question 7

What are the 4 most common underlying causes of PCT

Answer 7

Alcohol
VIral hepatitis
Oestrogens
Haemochromatosis

Question 8

What are the 2 aims of treatment of PCT

Answer 8

Relieve the skin disease and treat the underlying disease to reduce the risk of liver cirrhosis, hepatoma etc.

Question 9

What is the general underlying problem for Erythropoietic protoporphyria (EPP)

Answer 9

Genetic problem - single-nucleotide polymorphism

Question 10

What are the 2 main management techniques for EPP

Answer 10

6 monthly LFTs and RBC prophyrins

Visible ligt photo protection measures

Question 11

What are 4 examples of photo protection measures?

Answer 11

Behavioural - avoid mid - day sun
Clothing - importance of weave, colour etc.
Environmental - shade, window films
Topical sunscreen