ofg

Question 1

What is OFG?

Answer 1

Clinical presentation of oedema in the oral and facial soft tissues by blockage of lymphatic drainage due to an immune reaction

Question 2

What is angio-oedema?

Answer 2

Oedema of the oral and facial soft tissues due to an increase in fluid exudate from the capillaries but with no lymphatic drainage
Swelling comes up quickly and settles within 24-48 hours

Question 3

Describe the swelling present in OFG?

Answer 3

Swelling comes up quickly and settles very slowly over weeks and months
Often there is continuous swelling which changes in intensity from day to day

Question 4

Give 3 examples of other granulomatous diseases?

Answer 4

Sarcoidosis
Tuberculosis
Crohn’s disease

Question 5

What is the histological cause of swelling in OFG?

Answer 5

Giant cells forming within tissues which are obstructing the lymphatics

Question 6

What are the clinical signs of OFG?

Answer 6

Irritation and infection at the angles of the mouth
Lip fissuring
Crusty lips

Question 7

What are the 4 types of hypersensitivity reaction?

Answer 7

Type I - allergy and atopy
Type II - antibody-mediated hypersensitivity
Type III - immune complex-mediated hypersensitivity
Type IV - delayed type hypersensitivity

Question 8

Describe the type IV hypersensitivity reaction in OFG

Answer 8

T cells are activated by allergens which trigger macrophages to become active
The macrophages will try to phagocytose the allergen
If this isn’t possible the macrophages will fuse into the multinucleated giant cells (MGC)
Giant cells will then try to phagocytose and remove then antigen which may not always be possible

Question 9

When is OFG more likely to present?

Answer 9

Commoner in later childhood and adolescence

Question 10

What test is used form Crohn’s screening?

Answer 10

Faecal calprotectin

Question 11

When is faecal calprotectin useful?

Answer 11

Children under 7

Question 12

How is OFG managed?

Answer 12

Exclusion diet
Consider whether it is OFG or a part of Crohn’s disease by looking at GI symptoms, faecal calprotectin and sequential growth monitoring

Question 13

What foods should be avoided in an OFG exclusion diet trial?

Answer 13

Benzoin acid
Scorbic acid
Cinnamon products
Chocolate
E210-219

Question 14

How is OFG treated medically?

Answer 14

Topical miconazole for angular cheilitis, tacrolimus ointment for lip swelling and facial erythema
Intralesional steroid injection - triamcinolone
Systematic treatment to help immune modulation - azithryomycin, systemic immune modulation