OFG Flashcards

1
Q

What is OFG?

A

Oedema in the oral and facial soft tissues by blockage of lymphatic drainage due to an immune reaction.

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2
Q

What is angio-oedema?

A
  • Angio-oedema- increased vascular permeability and leaking into surrounding soft tissues (sudden increase in fluid overwhelms lymphatic drainage resulting in ballooning of tissues)
  • Fluid present within connective tissue
  • Happens quickly in an hour- settles within 24-48 hours
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3
Q

How does OFG differ from angio-oedema?

A

There is no excess exudate from capillaries into tissue
- Obstruction to lymphatic drainage occurs and accumulates causing swelling (caused by giant cells)
- Can happen quickly
- Settles slowly
- Triggers can be present all the time- so can last months and years (intensity of condition will vary)

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4
Q

What other disease could be responsible for granulomatous effects in face and mouth?

A
  • Sarcoidosis- LN and salivary glands (more common in pulmnory system)
  • Tuberculosis
  • Crohns- can affect any part of GI tract

-> all associated with giant cell production

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5
Q

What are the oral signs of OFG?

A
  • Less swelling than angio-oedema
  • Immune reactions- irritation at angles of mouth (angular cheilitis)
  • Lip fissuring
  • Crusting
  • Erythema of peri-oral tissues (can go to eye and ear)
  • Swelling of lips
  • Full thickness gingivitis (starts off as erythema)- initially not plaque related but as false pockets emerge it can become plaque associated
  • Swelling in floor of mouth at SM duct area (stag horning)
  • Tags of mucosa
  • Ulceration can be present- linear at depth pf sulcus (filled with granulomas on biopsy)
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6
Q

What occurs as a result of T1 hypersensitivity and what disease is it associated with?

A

Degranulation of mast cells in response to allergen- granules send vasoactive compounds into tissues which act on local blood vessels and increase vascular permeability and fluid exudate

-> Seen in Angio-oedema

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7
Q

What are the other types of hypersensitivity reactions?

A

T2- antibody mediated
T3- immune complex
T4- delayed (t cells are activated by allergen then trigger activation of macrophages)

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8
Q

What occurs in T4 hypersensitivity that can result in OFG?

A

T helper cells are activated and interact with/activate macrophages which attempt to phagocytose the allergen

If this is not possible- macrophages fuse to give giant cells (multinucleated) which try to phagocytose allergen (not always possible)

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9
Q

When does OFG present?

A

Can be any age- more common in children and adolescents

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10
Q

What is the severity of OFG for patients?

A

Some patients are unaware
-> mostly mild and controlled simply

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11
Q

What are the issues with severe unremitting form of OFG?

A

Reduced QoL

Appearance issues
-> may hamper child development
-> if extreme can lead to depression and prevent social contact

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12
Q

How is crohn’s screening carried out in children?

A
  • Look out for abdominal pain and altered bowel habit- ensure patients parents keep eye on this
  • Do not do colonoscopy unless evidence of GI disease
  • Measure Faecal Calprotectin- marker for inflammatory bowel change (if over 7)
  • Measure height and weight- should follow centile lines (if they move from one to the other it implies that there is growth, nutritional and bowel issues)
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13
Q

What is involved in initial management of OFG?

A

Consider whether true Oro-facial or related to Crohn’s
-> if crohn’s carry out screening

Diet history
-> identify overuse of allergens
-> consider exclusion diet trial

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14
Q

What foods are involved in exclusion diet for OFG?

A

Benzoic acid
-> found in carbonated drinks, can be replaced with water
-> remind patient this is found in many tomato based sauces

Sorbic acid

Cinnamon products

Chocolate

E210-219 where not included in the above

-> 100% compliance required for 3 months

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15
Q

What can be used to ensure exclusion diet is stuck to?

A

Food maestro- barcode scanning system to identify if allergens are present

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16
Q

What medical therapy is available for OFG patients who do not respond to exclusion diet?3

A

Miconazole/hydrocortisone for angular cheilitis

Tacrolimus ointment (0.03%)- absorbed into tissues into lymphatics and can help to disperse giant cells

Intra-lesional steroids- for areas of persisting swelling (may only last 6 months)

Immune suppressants
-> prednisolone pulses (check response of tissues)
-> If prolonged consider azathioprine, mycophenolate, adalimumab

17
Q

Where is OFG managed?

A

Manage in primary care as far as possible (symptomatic treatment)
-> If complex, refer