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.

OFG swelling comes up quickly and settles only very slowly – over weeks or months if at all.
* Often there is continuous swelling which changes in intensity from day to day or week to week – drainage is the issue
* No inc in exudate of fluid from capillaries into tissue
* There is an obstruction to lymphatic drainage – so accumulates in soft tissue = swelling

Granulomas in the tissue

Question 2

OFG vs angio-odema

Answer 2

Angio-oedema is oedema of the oral and facial soft tissues due to an INCREASE in fluid exudate from the capillaries but with NO lymphatic drainage
* comes up quickly and settles quickly – usually in 24-48 hours

OFG swelling comes up quickly and settles only very slowly – over weeks or months if at all.
* Often there is continuous swelling which changes in intensity from day to day or week to week – drainage is the issue
* No inc in exudate of fluid from capillaries into tissue, but there is an obstruction to lymphatic drainage – so accumulates in soft tissue = swelling
Granulomas in the tissue

Question 3

3 other granulomatous diseases

produce tissue giant cells

Answer 3

Sarcoidosis - rare

Tuberculosis - rare

Crohn’s Disease -common
* Can be hard to differentiate when looking at Crohn’s Vs OFG

Question 4

3 other granulomatous diseases

produce tissue giant cells

Answer 4

Sarcoidosis - rare

Tuberculosis - rare

Crohn’s Disease -common
* Can be hard to differentiate when looking at Crohn’s Vs OFG

Question 5

oro-facial granulomatosis clinical and histopathological findings

Answer 5

Less evidence of tissue swelling

Giant cells form within the tissue (multinucleated)
* obstructed lymphatics

More like an immune reaction seen around the mouth
* Lip fissuring, irritation/infection at angles, crusting.

quick onset, slow to settle

Question 6

type I hypersensitivity

Answer 6

angio-odema
* Degranulation of mast cells in response to an allergen
* Granules send vasoactive compounds into tissue to act upon BV to increase vascular permeability so inc fluid exudate

Question 7

type II hypersensitivity

Answer 7

antibody mediated hypersensitivity

Question 8

type III hypersensitivity

Answer 8

immune complex hypersensitivity

Question 9

type IV

Answer 9

Delayed type reaction
T cell activated by allergen which trigger macrophages to become active

Question 9

multinuclear giant cells
role

Answer 9

try to phagocytose and remove the allergen

not always possible
e.g. OFG and Crogns - difficulty so see many multinucleate giant cells in histology of biopsy

Question 10

OFG group affected

Answer 10

Common in later childhood and adolescent

Often present at a low level – patient unaware of the changes
Most are mild and can be controlled with simple measures

Severe and unremitting forms can be extremely disabling for the patient
Significant reduction in their QoL

Issues with appearance at a very important part of a child’s development
* Often will lead to depression and withdrawal from social contact when extreme

Question 11

OFG signs/symptoms

8

Answer 11

Significant erythema of perioral tissues – disease extending well out from the mouth – can be up to eye or ear

Swelling of lip(s)

Change in skin quality

Angular cheilitis

Fissuring of lips

Full thickness gingivitis
* Starts of erythema, doesn’t cause any change in size of gingiva – but progresses with time
* Not plaque induced but can become plaque associated as false pocketing develops with gingival enlargement

Swelling in of intra oral mucosa
* FOM – submandibular duct area enlarged
* Buccal mucosa
* small tags can form anywhere

Ulceration
* Linear ulcer in depth of sulcus
* If biopsied – filled with granulomas
* Not associated with aphthous ulcer triggers (e.g. def) but is with granulomatosis diseases

Question 12

Crohn’s screening

Answer 12

Parental awareness of importance of altered bowel habit or abdominal pain

Growth monitoring at each hospital visit
* Plotted onto chart – should follow a centile line
* If across = problem with growth/nutritional

Faecal Calprotectin assay
* Unreliable in younger children (7+)
* Screening test for endoscopy - Marker for inflammatory bowel changes
* Good predictor of Crohn’s disease activity

Invasive investigation – when evidence of GI disease

Question 13

when does invasive (endoscopy) investigation for crohns happens

Answer 13

when evidence of GI disease

faecal calprotectin

Question 14

management of OFG

primary care
3

Answer 14

3 month empirical dietary exclusion
* Benzoate/cinnamon – unless clear other dietary triggers

Topical treatment to angular chelitis/fissure
* Miconazole/hydrocortisone cream

Topical treatment to lip swelling or facial erythema
* Tacrolimus ointment 0.03%
* Intralesional steroids to lip
* Systemic immune modulation?

Question 15

initial management of OFG

Answer 15

Consider whether this is Oro-facial or part of Crohns
* GI symptoms
* Faecal calprotectin
* Start sequential growth monitoring

Take dietary history
* Identify ‘overuse’ of dietary allergens
* Discuss the role of a complete exclusion diet trial

Exclusion diet trial (primary care or specialist)

Question 16

exclusion diet for OFG

Answer 16

Avoid all foods containing
* Benzoic acid
* Sorbic acid
* Cinnamon products
* Chocolate
* E210-219 where not included in the above

Support with dietetics or other aids as needed (food maestro app)

100% compliance for 3 months
* If helpful then test re-introduction of ‘favourite’ excluded foods
* See if any trigger OFG – yes, then personal preference whether they have it or not

No other reliable tests for dietary allergens other than exclusion diet to see if trigger OFG (Skin testing not reliable)

Benzoates found in tomato and tomato products
* All things with tomato sauces must be avoided

Question 17

Medical tx for OFG

speciliast only
3

Answer 17

Topical treatment
* Miconazole to angular chelitis
* Tacrolimus ointment to areas of lip swelling and facial erythema – g*oes to lymphatics to aid dispersal of obstructing giant cells *

Intralesional steroid injection
* Triamcinolone injected into the area of swelling – often weekly for 3 weeks, only useful for 6 months

Systemic treatment to help immune modulation
* Pulsed azithromycin for 3 months – 3 days in every week
Systemic immune modulation
* Prednisolone pulse for short term issues – assess responsiveness of tissues
* Azathioprine/mycophenolate for prolonged therapy