11 - Autoimmune Diseases of Mucous Membranes and Necrotizing Periodontitis Flashcards

1
Q

what are diseases presenting as desquamative gingivitis

A
  1. Lichen Planus
  2. Cicatricial Pemphigoid
  3. Pemphigus Vulgaris
  4. Linear IgA Disease
  5. Lupus Erythematosus
  6. Erythema Multiforme
  7. Drug Eruptions
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2
Q

is desquamative gingivitis a clinical term or a specific diagnosis?

A

CLINICAL TERM ONLY!
NOT a specific diagnosis!

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3
Q

what is sloughing of the gingiva

A

desquamative gingivitis

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4
Q

75% of desquamative gingivitis cases have a ___ basis

A

dermatologic basis

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5
Q

what is the first sign of desquamative gingivitis

A

oral signs

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6
Q

who most commonly gets desquamative gingivitis

A

women in 4th-5th decades

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7
Q

desquamative gingivitis symptomatic or asymptomatic

A

may be asymptomatic

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8
Q

describe desquamative gingivitis

A
  • Mild burning to intense pain
  • Usually confined to the gingiva
  • May involve other intraoral or extra-oral sites
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9
Q

how do you obtain final diagnosis of desquamative gingivitis

A

biopsy

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10
Q

steps for reaching final diagnosis for desquamative gingivitis

A
  1. Clinical History
  2. Clinical Examination
  3. Biopsy
  4. Microscopic Examination
  5. Immunofluorescence
  6. Management
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11
Q

what do you look for when searching clinical history when reaching for final diagnosis of desquamative gingy cases

A
  • Medical history - Medications
  • Symptoms
  • When did the lesion start?
  • Has it worsened?
  • What exacerbates the condition? - Foods, mouthrinses, toothpaste, brushing, habits
  • Previous therapy
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12
Q

what is blister formation or peeling of skin/mucosa when horizontal tangential pressure is applied

A

Nikolsky’s sign

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13
Q

what biopsy:

portion of lesion is removed

A

incisional biopsy

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14
Q

what biopsy:

entire lesion is removed in biopsy

A

excisional biopsy

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15
Q

include ___ tissue in the biopsy

A

perilesional (means somewhat normal looking tissue)

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16
Q

rules for perilesional tissue in biopsy

A
  • Need intact epithelium in the biopsy
  • Avoid areas of ulceration
  • Intact epithelium is needed to make a histological diagnosis
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17
Q

what type of biopsy

A

tissue punch biopsy

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18
Q

what is Michel’s solution/buffer

A

ammonium sulfate (pH 7.0)

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19
Q

what is done for immunofluorescene assessment

A
  • Place one biopsy in Formalin for H&E stain
  • Place one biopsy in Michel’s Buffer (ammonium sulfate, pH 7.0) for Immunofluorescence Assessment
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20
Q

what to do in microscopic exam

A
  • Conventional H&E stain
  • Direct Immunofluorescence (DIF)
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21
Q

what is direct immunofluorescene

A
  • Specimen incubated with fluorscein- labeled antihuman serum
  • Anti-IgA, anti-lgM, anti-lgG, antifibrin, and anti-C3
  • Binds to antibodies in the specimen and creates fluorescence
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22
Q

what is:

Inflammatory mucocutaneous disease
Can involve: Oral cavity, genital tract, skin, scalp, nails

Prevalence: 0.1% - 4%
Majority of patients are female aged 40’s-50’s
2:1 Female-Male ratio

A

lichen planus

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23
Q

is lichen planus T or B cell mediated

A

T cell

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24
Q

1% of oral lichen planus may develop what

A

SCC

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25
Q

clinical forms of lichen planus? what is the most common?

A

Bullous
Reticular - most common
Erosive - most common
Atrophic
Patch

BREAP

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26
Q

where are lichen oral lesions present? unilateral or bilateral?

A

mostly bilateral!
Buccal mucosa, Tongue, Lateral border, dorsum, Hard palate, Alveolar ridge, Gingiva

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27
Q

what is:

Usually asymptomatic
Bilateral
Lacy white lines
Wickham’s Striae

A

reticular lichen planus

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28
Q

what is:

Erythematous
Pain
Ulcerated areas
Sensitive to heat, acid, spicy foods

A

erosive lichen planus

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29
Q

what is:
Erythematous
Pain
Ulcerated areas
Sensitive to heat, acid, spicy foods

A

erosive lichen planus gingival lesions

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30
Q

what does lichen planus immunopathology look like

A
  • linear fibrin deposits along basement membrane zone
  • band-like infiltrate of T lymphocytes in lamina propria
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31
Q

how to tx asymptomatic lichen planus

A
  • do not require tx
  • follow up every 6-12 months
32
Q

tx of erosive lichen planus

A
  1. Topical steroids
    • 0.05% clobetasol gel/cream
    • 0.05% fluocinonide gel/cream
    • 0.1% tacrolimus ointment (for recalcitrant cases)
  2. Re-evaluate in 2 weeks
  3. Follow-up every 3-6 months
33
Q

sample rx of lichen planus

A

Lidex (0.05% fluocinonide) gel
Disp: One tube 1.5 g
Sig: Apply to affected area after meals and at
night time.

34
Q

tx of severe or refractory lichen planus

A
  1. Refer to oral pathologist and dermatologist
  2. Physician should prescribe systemic steroids
35
Q

what is cicatrical pemphigoid also called

A

Mucous Membrane Pemphigoid (MMP)

36
Q

cicatricial pemphigoid is what type of disorder? how does this occur?

A

vesiculobullous autoimmune disorder
occurs to due to antibodies to protein in the basement membrane

37
Q

who gets cicatricial pemphigoid? where?

A

Most common in women in 5th decade
Oral cavity
Extra-oral sites including conjunctiva of eye, genital tract

38
Q

describe ciciatricial pemphigoid ocular lesions

A
  1. Conjunctivitis
  2. Symblepharon- Adhesion of eyelid to
    eyeball
  3. Can lead to blindness
39
Q

what is the adhesion of eyelid to eyeball

A

symblepharon

40
Q

what is this? what is this at risk of in severe cases

A

cicatricial pemphigoid -> can lead to blindness in severe cases

41
Q

immunopathology of cicatricial pemphigoid

A

IgG and C3 at basement membrane on Direct Immunofluorescence

42
Q

what are the stains that can be used to diagnose cicatricial pemphigoid? what do you see

A
43
Q

how to treat ciciatricial pemphigoid

A
  1. Refer to ophthalmologist
  2. Topical steroids
    • 0.05% fluocinonide gel and 0.05% clobetasol propionate
  3. Severe cases
    • Refer to oral pathologist and dermatologist
    • Systemic steroids for ocular involvement
  4. Follow-up
44
Q

what is:

Autoimmune bullous disorder
Antibodies to cell surface glycoproteins on keratinocytes (epithelial cell)

Most common in women in 4th decade
Oral lesions frequently first sign
Potentially fatal disorder (10% mortality rate)

A

pemphigus vulgaris

45
Q

oral sites to find pemphigus vulgaris

A

Soft palate, buccal mucosa, tongue, labial mucosa, gingiva

46
Q

when looking at oral lesions, what two (without histological observance) looks similar

A

ciciatricial pemphigoid and pemphigus vulgaris

47
Q

what is:

Intercellular IgG and C3 deposits in the epithelium
Intraepithelial clefting

A

pemphigus vulgaris

48
Q

what are the stains to diagnose pemphigus vulgaris? what do you see?

A
49
Q

how to treat pemphigus vulgaris

A
  1. Immediate referral to dermatologist or rheumatologist
  2. Mycophenolate mofetil (CellCept®)- immunosuppressant
  3. Rituximab- monoclonal antibody against B cells (to prevent skin involvement and associated morbidity)
  4. Follow-up for response to treatment
50
Q

what is an uncommon mucocutaneous disorder with predilection for women?

A

Linear IgA disease

51
Q

etiology of Linea IgA

A

not fully understood BUT can be drug induced (ACE inhibitor- blood pressure medication)

52
Q

oral sites of linear IgA disease

A

palate, tonsillar pillars, buccal mucosa, tongue, gingiva

53
Q

extra-oral sites of linear IgA disease

A

Skin of upper and lower trunk, shoulders, limbs
Pruritic vesiculobullous rash

54
Q

histologically what does linear IgA disease show

A

Linear deposits of IgA along basement membrane

55
Q

linear IgA disease treatment

A
  1. Refer to dermatologist
  2. Sulfones and Dapsone
  3. Systemic steroids- small doses
56
Q

pemphigus vulgaris

A
57
Q

cicatricial pemphigoid

A
58
Q

lichen planus

A
59
Q

linear IgA disease

A
60
Q

what is:

Autoimmune disease
Antinuclear antibodies
Malar rash uncommon

A

SLE

61
Q

oral sites of SLE

A

Oral sites: Palate, buccal mucosa, lips, gingiva
Oral lesions look similar to lichen planus

62
Q

who gets SLE? what does it affect

A

Predilection for women (10:1)
Affects vital organs (kidneys, heart), skin,
mucosa

63
Q

SLE tx

A

Refer to dermatologist
Systemic steroids
Topical steroids

64
Q

what is:

  • Acute bullous and macular inflammatory mucocutaneous disease
  • Development of Immune complex vasculitis
  • Destruction of vascular walls, necrosis of epithelium and CT

May be life-threatening
Large painful ulcers

A

erythema multiforme

65
Q

what are target or iris lesions

A

erythema multiforme

66
Q

how does erythema multiforme affect oral mucosa

A

buccal mucosa, tongue, labial mucosa most common

67
Q

etiology of erythema multiforme

A

Herpes simplex infection
Drug reaction: Sulfonamides, Penicillins, Quinolones, Chlormezanone, Barbiturates, NSAIDS, Anticonvulsants, Allopurinol

68
Q

immunofluorescence of erythema multiforme

A

Negative
Rules out other vesiculobullous diseases

69
Q

tx if erythema multiforme

A

Antihistamines
Topical anesthetic, debridement of lesions
Some use of steroids

70
Q

what are drug eruptions

A

certain medications may cause oral lesions resembling lichen planus or vesiculobullous disease

71
Q

what are drug eruption diseases

A

Lichenoid Drug Eruptions
Lichenoid Mucositis

72
Q

what can cause drug eruptions

A
  1. medications
  2. cinnamon
  3. cinnamic aldehyde
73
Q

what is used to mask taste of pyrophosphates in tartar control toothpase

A

cinnamic aldehyde

74
Q

drugs associated with lichenoid drug eruptions

A
75
Q

tx of drug eruptions

A

discontinue offending agent

76
Q

what are differential diagnoses

A

Lichen Planus
Cicatricial Pemphigoid
Pemphigus Vulgaris
Linear IgA Disease
Lupus Erythematosus
Erythema Multiforme
Drug Eruptions