key points OFG

Question 1

definition

Answer 1

oedema in oral and facial STs by blockage of lymphatic drainage due to an immune reaction
- no increase in exudate of fluid from capillaries into tissues - obstruction of lymphatic drainage is the problem. Fluid accumulates

Question 2

onset and resolve

Answer 2

comes up quickly and settles only v slowly/not at all
often continuous swelling which changes in intensity
- trigger can be intermittent/constant

Question 3

histology

Answer 3

GCs which block lymphatic drainage
MN
oedema, dilated lymph

Question 4

angioedema definition

Answer 4

oedema of oral and facial STs due to increase in fluid exudate from capillaries (vascular permeability) but with no lymphatic drainage
comes up quickly and resolves quickly (24-48hrs)

Question 5

angioedema histology

Answer 5

fluid in CT bundles

Question 6

other granulomatous diseases producing tissue GCs

Answer 6

sarcoidosis
TB
Crohn’s

Question 7

clinical appearance

Answer 7

angular cheilitis
lip fissuring and crusting
erythema of perioral tissues
swelling of lips
full thickness gingivitis (starts as erythema) - false pockets
swelling of IO tissues
cobblestoning
staghorning - SM duct enlarged
mucosal tags
ulceration

Question 8

presentation age

Answer 8

any age - common later childhood and adolescent

Question 9

Crohn’s screening

Answer 9

parental awareness of importance of altered bowel habit/abdo pain
growth monitoring
faecal calprotectin assay

Question 10

faecal calprotectin assay

Answer 10

unreliable young kids
>7yrs good marker for inflammatory bowel changes
screening test for endoscopy
good predictor of Crohn’s disease activity

Question 11

T1 hypersensitivity - angioedema

Answer 11

degranulation of mast cells in response to an allergen
IgE
granules send vasoactive compounds into tissues, act upon local bv’s to increase vascular permeability and fluid exudates

Question 12

T4 hypersensitivity OFG

Answer 12

T cells activated by an allergen, trigger macrophages to become active
produce activated macrophages which try to phagocytose the allergen
if not possible, macrophages will fuse into MNGCs to try to phagocytose allergen
- difficulty in removing trigger
- induced by IL-4

Question 13

initial management

Answer 13

consider whether orofacial or part of Crohns
- GI symptoms, faecal calprotectin
- start sequential growth monitoring
take dietary history
- identify ‘overuse’ of dietary allergens
- discuss role of a complete exclusion diet trial

Question 14

exclusion diet

Answer 14

benzoic acid
sorbic acid
cinnamon products
chocolate
E210-219 where not inc in above

for 3m

Question 15

after exclusion diet

Answer 15

test reintroduction of fav foods

can then choose what foods they will inc etc

Question 16

is skin testing reliable?

Answer 16

no

Question 17

what are benzoates found in?

Answer 17

tomato and tomato based products

Question 18

medical therapy

Answer 18

topical
intralesional steroid injection
systemic tx

Question 19

topical tx

Answer 19

miconazole/hydrocortisone to angular cheilitis
tacrolimus ointment 0.03% to lip swelling and facial erythema
- absorbed and carried to local lymphatics where it can help to disperse obstructing GCs

Question 20

intralesional steroid injection

Answer 20

triamcinolone injected into area of swelling - often weekly for 3wks
often needs repeating - every 6m

Question 21

systemic tx

Answer 21

pulsed azithromycin 3m - 3 days every week
systemic immune modulation
- prednisolone pulse for short term issues
- azathioprine/mycophenolate
biologics have been used w some success