Orofacial Granulomatosis Flashcards
What is Orofacial Granulomatosis?
Clinical presentation of oedema in the oral and facial soft tissues by blockage of lymphatic drainage due to an immune reaction
How does Orofacial Granulomatosis occur?
It is due to an increase in fluid exudate from the capillaries but with no lymphatic drainage
There is an increased vascular permeability which allows fluid to leak from the vessels and into the soft tissues
This sudden increase overwhelms the drainage of the tissues through the lymphatics and the tissues balloon up
What dietary allergens are associated with OFG? How are these detected
Benzoate, Sorbate, Cinnamon, Chocolate
Use dietary exclusion to determine trigger
Skin testing is not reliable
What are some symptoms of OFG?
Swelling that comes on quickly and settles very slowly (over weeks and months)
Often there is continuous swelling which changes in intensity from day to day or week to week depending on the trigger
Irritation at the angles of the mouth
Lip fissuring and crusting
What is the management of OFG?
3 month empirical dietary exclusion
–benzoate/cinnamon unless clear other triggers
Topical treatment for angular cheilitis/fissure
–miconazole/hydrocortisone cream
Topical treatment to lip swelling or facial erythema
–Tacrolimus ointment 0.03%
–systemic immune modulation (pulsed azithromycin for 3 months 3 days a week, prednisolone pulse for short term issues, Azathioprine/mycophenolate for prolonged therapy
Intralesional steroid injection
–Triamcinolone injected into the area of swelling- often weekly for 3 weeks
What is the initial management of OFG?
Consider whether this is Oro-facial or part of Crohn’s
–GI symptoms
–Faecal calprotectin
–Start sequential growth monitoring
Take dietary history
–Role of a complete exclusion diet trial to see if reducing intake of allergens reduces symptoms
What is Type IV hypersensitivity?
Cell mediated
Delayed type reaction where T cells are activated by an allergen and trigger macrophages to become active which will try to phagocytose the allergen. If this is not possible the macrophages fuse into multinucleated giant cells and try to phagocytose to remove the allergen
What is Type I Hypersensitivity?
Present in angio-oedema where we have degranulation of mast cells due to an allergen and these granules send vasoactive compounds into the tissues where they act on the local blood vessels to increase vascular permeability and therefore fluid exudate
