Orofacial granulomatosis

Question 1

What is Orofacial granulomatosis?

Answer 1

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

Question 2

What is angio-oedema?

Answer 2

• Oedema of the oral and facial soft tissues due to increase in fluid exudate from capillaries but with no lymphatic drainage

Question 3

What is the difference between angio-oedema and OFG?

Answer 3

• OFG blockage of lymphatic drainage
• OFG swelling comes up quick and settles slow (over weeks or months if at all)
• Angio swelling comes up quick and settles quickly usually in 24-48hrs

Question 4

What are some diseases other than OFG than can be responsible for swelling in facial or oral areas?

Answer 4

• Produce giant cells responsible for the granulomatosis
• Sarcoidosis (rare)
• Tuberculosis (rare)
• Crohns disease

Question 5

What is this picture showing us?

Answer 5

• Angio-oedema
• Can see lots of fluid present within the tissue leading to facial swelling

Question 6

What disease is this picture presenting as?

Answer 6

• Orofacial granulomatosis
• Giant cells forming within the tissues preventing lymphatic drainage
• Can see the multinucleated cells within the membrane

Question 7

What is going on during OFG histologically and immunologically speaking?

Answer 7

• Type IV ( Cell mediated) hypersensivity
• Bacteria or antigen trigger an immune response
• Antigen presenting cells activate the T helper cells (TH1)
• T helper cells bind to macrophages to produce activated machrophages which will try and phagocytose the antigen.
• Macrphages fusion induced by IL-4 occurs to produce multinucleated giant cell which also try to phagocytose the antigen

Question 8

What age does OFG present?

Answer 8

• Can present any age
• Common in later childhood and adolescent
• Most are mild and controlled with simple measures and pt unaware

Question 9

What clinical findings can be seen with OFG?

Answer 9

• Perioral erythema
• Swelling of lips
• Angular chelillits
• Fissuring of lips
• Full thickness gingivitis (not plaque related initially)
• Swelling of intra oral tissues
• Ulceration (usually filled with granulomas histologically)

Question 10

What should the parents be warned about if the child has OFG?

Answer 10

• Should undergo a Chrohns screening
• Parents be aware of altered bowel habit or abdominal pain
• Growth monitoring at each hospital visit

Question 11

What is the best way to measure Chrohsns in child if diagnosed with OFG?

Answer 11

• Faecal Calprotectin assay
• Good predictor of Crohns disease activity
• Screening test for endoscopy
• Unreliable in children

Question 12

When initially manging diagnosis of OFG for a child what is the first port of call?

Answer 12

• Consider whether this if OFG or part of Crohns disease
• Check GI symptoms
• Faecal calprotectin
• Start sequential growth monitoring
• Take diet history and identify overuse of dietary allergen

Question 13

What does the Exclusion Diet trail entail?

Answer 13

• Avoid all food containing
• Benzoic acid
• Sorbic acid
• Cinnamon products
• Chocolate
• E210-219
• Compliance needs to be 100% for 3 months

Question 14

What medical therapy can be used when diet testing is not useful? (Specialist only)

Answer 14

Topical txt
- Miconazole to angular chelitis
- Tacrolimus ointment to areas of lip swelling and facial erythema

Intralesional steroid injection
- Triamcinolone injected into area of swelling - often weekly for 3 weeks

Systemic txt to help immune modulation
- Pulse azithromycin for 3 months 3 days in every week
- Prednisolone pulps for short term issues
- Azathioprine/mycophenolate for longer term