7. Systemic Disease (inc. Orofacial Granulomatosis) Flashcards

1
Q

What are the three main causes of dental manifestations of systemic disease in children ?

A

Congenital conditions/infections.
Illness/metabolic disorder.
Pigmentation from blood substances.

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2
Q

Name 3 congenital conditions/infections which might cause dental manifestations in children.

A

Syphilis.
TORCH.
Ectodermal dysplasia.

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3
Q

Name two pigmentation substances which might cause dental manifestations in children.

A

Bilirubin.
Tetracycline.

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4
Q

What gene is ectodermal dysplasia commonly associated with ?

A

EDA

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5
Q

What is ectodermal dysplasia ?

A

Genetic defect (gene mutation of EDA gene) causing disorders affecting skin, sweat glands, hair, teeth, immune system, hearing, vision.

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6
Q

What can be the 3 dental manifestations of ectodermal dysplasia ?

A

Small cone shaped teeth.
Hypodontia.
Small molar teeth.

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7
Q

What can be the 4 dental manifestations of syphilis infection passed from mother to child during pregnancy ?

A

Hutchison’s incisors - bulbous crowns.
Moon’s molars.
Fournier’s molars.
Enamel hypoplasia.

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8
Q

What teeth are most commonly affected by congenital syphilis infection ?

A

Permanent central incisors.
Mandibular first molars.

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9
Q

What is porphyria and how does it affect tooth development ?

A

Inherited metabolic condition caused by gene mutation.
Produces a change in amount of haem and haem products in the blood.
Causing dark pigmentation incorporated into tooth structure.

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10
Q

What colour of discolouration will high bilirubin levels (due to jaundice) cause the tooth to develop ?

A

Yellow/green.

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11
Q

What colour of discolouration will tetracycline treatment cause tooth to develop ?

A

Linear band of grey discolouration.

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12
Q

Name oral mucosal effects from systemic disease.

A

Giant cell granuloma.
OFG.
Recurrent aphthous stomatitis.
Dermatoses - lichen planus and vesiculobullous conditions.
Immune deficiency/disease.
Drug interactions.

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13
Q

What are the causes of giant cell lesions ?

A

Irritation and PTH excess.

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14
Q

Why might giant cell lesions be caused by excess PTH ?

A

Excess parathyroid stimulation of osteoclasts.
Causes loss of cortical bone - densest bone with highest calcium content.

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15
Q

Name two reactive parathyroid conditions.

A

Renal failure.
Hypocalcaemia.

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16
Q

What are the dental radiographic effects of ongoing hyperparathyroidism ?

A

Loss of lamina dura - might look like PA lesion, will be absent surrounding the root (not only apex).
When hyperparathyroidism is treated - lamina dura will reform.

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17
Q

What is the dental consequences of raised ACTH levels ?

A

Widespread hyperpigmentation of mucosa.
Due to increased melanocyte stimulation hormone being produced.

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18
Q

What are the potential causes of raised ACTH levels in the body ?

A

Addison’s disease.
Cushing’s disease (where pituitary adenoma is producing excess ACTH).
Small cell carcinoma in the lung (where ACTH is produced inappropriately).

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19
Q

What dental conditions can patients with immune deficiency/disease be predisposed to ?

A

OFG.
Sjorgen’s syndrome.
Fungal and viral infection.

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20
Q

What skin conditions can be reflected in the oral mucosa ?

A

Pemphigoid.
Pemphigus.
Lichen planus.

21
Q

What are the potential causes for hematinic deficiencies ?

A

Bleeding.
Diet.
Increased demand.
Malabsorption.

22
Q

What are the dental consequences of hematinic deficiency ?

A

Oral ulceration.
Painful tongue.

23
Q

What are some of the symptoms/signs of OFG ?

A

Perioral erythema and swelling.
Lip swelling.
Angular chelitis.
Oedema of floor of the mouth (stag horning).
Linear fissured ulcer between attached and unattached mucosa in anterior buccal sulcus.
Proliferative, erythematous, full thickness gingivitis with no plaque responsible (or disproportionate reaction) and present in all quadrants.

24
Q

What tests can be used to screen for Crohn’s disease ?

A

Faecal calprotectin assay.
This will indicate need for endoscopy.

25
Q

What is faecal calprotectin assay ?

A

Test for inflammatory proteins.

26
Q

OFG - how should associated angular cheilitis be managed ?

A

Miconazole/hydrocortisone cream.

27
Q

OFG - how should associated lip swelling or facial erythema be managed ?

A

Tacrolimus ointment 0.03%.
Topical steroids - miconazole/hydrocortisone cream.
Intralesional steroids to lip.
Systemic immune modulatory treatment.

28
Q

Name 4 CT autoimmune conditions.

A

Systemic lupus erythematosus (SLE)
Systemic sclerosis (scleroderma)
Sjogren’s syndrome
Mixed CT disease (MCTD)

29
Q

What oral condition can lupus present similarly to ?

A

Lichen planus.

30
Q

When should you be suspicious of lupus of a lesion which looks similar to lichen planus ?

A

When found on keratinized tissue i.e. hard palate.

31
Q

What tests can you carry out to determine lupus vs. lichen planus ?

A

Immunology assays of the blood.
Histological sample - lymphocytic band will be lower placed in CT in lupus, compared to lichen planus.

32
Q

What are the dental implications of systemic sclerosis ?

A

Hemangiomas can cause mucosal bleeding in oral cavity.
Perioral fibrosis causing limited mouth opening.

33
Q

What is Wegener’s granulomatosis ?

A

Small vessel vasculitis disease - vasculitis change of gingivae and palate causing ischemia and necrosis of tissue.
Risk of spread into upper airway. Medical emergency - requires urgent referral to rheumatology for systemic immunosuppression.

34
Q

What are the oral effects of immune deficiency ?

A

Risk of oral opportunistic infection.
Sjorgen’s syndrome.
HIV related salivary disease.
Kaposi’s sarcoma.
Hairy leukoplakia.
Viral herpetic reactivation ulceration.

35
Q

What are the four causes of haematinic deficiencies ?

A

Poor intake.
Malabsorption.
Blood loss.
Increased demand.

36
Q

What drug group pose risk of osteonecrosis ?

A

Bisphosphonates.

37
Q

What drug groups pose risk of angio-oedema ?

A

ACE inhibitors.

38
Q

What drug groups pose risk of lichenoid reactions ?

A

ACE inhibitors and beta blockers.

39
Q

What drug groups pose risk of oral ulceration ?

A

Beta blockers and NSAIDs.

40
Q

What drug groups pose risk of xerostomia ?

A

Tricyclic antidepressants and anti-cholinergic drugs.

41
Q

What are the key differences between oral ulceration and drug induced oral ulceration ?

A

Drug induced - will not have erythematous change and will not respond to topical steroids. Will only resolve when removal of drug causing condition.

Normal - will have erythematous halo surrounding ulcer and will respond to topical steroids.

42
Q

What is angio-oedema ?

A

Rapid swelling of the face within an hour caused by INCREASE in fluid exudate from capillaries but with no lymphatic drainage.

43
Q

Define OFG.

A

Clinical presentation of oedema in oral and facial soft tissue by blockage of lymphatic drainage (by granulomas) due to an immune reaction causing accumulation of fluid in tissues.

44
Q

What type of hypersensitivity reaction is angio-oedema ? And explain.

A

Type 1 -
Degranulation of mast cells in reaction to an allergen.
Causing release to vasoactive compounds which cause local blood vessels to increase permeability.

45
Q

What is type 2 hypersensitivity ?

A

Antibody mediated hypersensitivity.

46
Q

What is type 3 hypersensitivity ?

A

Immune complex hypersensitvity.

47
Q

What type of hypersensitivity reaction is OFG ? And explain.

A

Type 4 -
Delayed reaction - T cell activated by allergen triggering macrophages to become active and attempt to phagocytose allergen.
Where this is not possible, macrophages fuse to form multinucleate giant cells.

48
Q

What age are you most likely to be affected by OFG ?

A

Later childhood and adolescent.