11 - Autoimmune Diseases of Mucous Membranes and Necrotizing Periodontitis Flashcards
1
Q
what are diseases presenting as desquamative gingivitis
A
- Lichen Planus
- Cicatricial Pemphigoid
- Pemphigus Vulgaris
- Linear IgA Disease
- Lupus Erythematosus
- Erythema Multiforme
- Drug Eruptions
2
Q
is desquamative gingivitis a clinical term or a specific diagnosis?
A
CLINICAL TERM ONLY!
NOT a specific diagnosis!
3
Q
what is sloughing of the gingiva
A
desquamative gingivitis
4
Q
75% of desquamative gingivitis cases have a ___ basis
A
dermatologic basis
5
Q
what is the first sign of desquamative gingivitis
A
oral signs
6
Q
who most commonly gets desquamative gingivitis
A
women in 4th-5th decades
7
Q
desquamative gingivitis symptomatic or asymptomatic
A
may be asymptomatic
8
Q
describe desquamative gingivitis
A
- Mild burning to intense pain
- Usually confined to the gingiva
- May involve other intraoral or extra-oral sites
9
Q
how do you obtain final diagnosis of desquamative gingivitis
A
biopsy
10
Q
steps for reaching final diagnosis for desquamative gingivitis
A
- Clinical History
- Clinical Examination
- Biopsy
- Microscopic Examination
- Immunofluorescence
- Management
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
12
Q
what is blister formation or peeling of skin/mucosa when horizontal tangential pressure is applied
A
Nikolsky’s sign
13
Q
what biopsy:
portion of lesion is removed
A
incisional biopsy
14
Q
what biopsy:
entire lesion is removed in biopsy
A
excisional biopsy
15
Q
include ___ tissue in the biopsy
A
perilesional (means somewhat normal looking tissue)
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
17
Q
what type of biopsy
A
tissue punch biopsy
18
Q
what is Michel’s solution/buffer
A
ammonium sulfate (pH 7.0)
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
20
Q
what to do in microscopic exam
A
- Conventional H&E stain
- Direct Immunofluorescence (DIF)
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
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
23
Q
is lichen planus T or B cell mediated
A
T cell
24
Q
1% of oral lichen planus may develop what
A
SCC
25
clinical forms of lichen planus? what is the most common?
Bullous
Reticular - most common
Erosive - most common
Atrophic
Patch
BREAP
26
where are lichen oral lesions present? unilateral or bilateral?
mostly bilateral!
Buccal mucosa, Tongue, Lateral border, dorsum, Hard palate, Alveolar ridge, Gingiva
27
what is:
Usually asymptomatic
Bilateral
Lacy white lines
Wickham's Striae
reticular lichen planus
28
what is:
Erythematous
Pain
Ulcerated areas
Sensitive to heat, acid, spicy foods
erosive lichen planus
29
what is:
Erythematous
Pain
Ulcerated areas
Sensitive to heat, acid, spicy foods
erosive lichen planus gingival lesions
30
what does lichen planus immunopathology look like
- linear fibrin deposits along basement membrane zone
- band-like infiltrate of T lymphocytes in lamina propria
31
how to tx asymptomatic lichen planus
- do not require tx
- follow up every 6-12 months
32
tx of erosive lichen planus
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
sample rx of lichen planus
Lidex (0.05% fluocinonide) gel
Disp: One tube 1.5 g
Sig: Apply to affected area after meals and at
night time.
34
tx of severe or refractory lichen planus
1. Refer to oral pathologist and dermatologist
2. Physician should prescribe systemic steroids
35
what is cicatrical pemphigoid also called
Mucous Membrane Pemphigoid (MMP)
36
cicatricial pemphigoid is what type of disorder? how does this occur?
vesiculobullous autoimmune disorder
occurs to due to antibodies to protein in the basement membrane
37
who gets cicatricial pemphigoid? where?
Most common in women in 5th decade
Oral cavity
Extra-oral sites including conjunctiva of eye, genital tract
38
describe ciciatricial pemphigoid ocular lesions
1. Conjunctivitis
2. Symblepharon- Adhesion of eyelid to
eyeball
3. Can lead to blindness
39
what is the adhesion of eyelid to eyeball
symblepharon
40
what is this? what is this at risk of in severe cases
cicatricial pemphigoid -> can lead to blindness in severe cases
41
immunopathology of cicatricial pemphigoid
IgG and C3 at basement membrane on Direct Immunofluorescence
42
what are the stains that can be used to diagnose cicatricial pemphigoid? what do you see
43
how to treat ciciatricial pemphigoid
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
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)
pemphigus vulgaris
45
oral sites to find pemphigus vulgaris
Soft palate, buccal mucosa, tongue, labial mucosa, gingiva
46
when looking at oral lesions, what two (without histological observance) looks similar
ciciatricial pemphigoid and pemphigus vulgaris
47
what is:
Intercellular IgG and C3 deposits in the epithelium
***Intraepithelial*** clefting
pemphigus vulgaris
48
what are the stains to diagnose pemphigus vulgaris? what do you see?
49
how to treat pemphigus vulgaris
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
what is an uncommon mucocutaneous disorder with predilection for women?
Linear IgA disease
51
etiology of Linea IgA
not fully understood BUT can be drug induced (ACE inhibitor- blood pressure medication)
52
oral sites of linear IgA disease
palate, tonsillar pillars, buccal mucosa, tongue, gingiva
53
extra-oral sites of linear IgA disease
Skin of upper and lower trunk, shoulders, limbs
Pruritic vesiculobullous rash
54
histologically what does linear IgA disease show
Linear deposits of IgA along basement membrane
55
linear IgA disease treatment
1. Refer to dermatologist
2. Sulfones and Dapsone
3. Systemic steroids- small doses
56
pemphigus vulgaris
57
cicatricial pemphigoid
58
lichen planus
59
linear IgA disease
60
what is:
Autoimmune disease
Antinuclear antibodies
Malar rash uncommon
SLE
61
oral sites of SLE
Oral sites: Palate, buccal mucosa, lips, gingiva
Oral lesions look similar to lichen planus
62
who gets SLE? what does it affect
Predilection for women (10:1)
Affects vital organs (kidneys, heart), skin,
mucosa
63
SLE tx
Refer to dermatologist
Systemic steroids
Topical steroids
64
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
erythema multiforme
65
what are target or iris lesions
erythema multiforme
66
how does erythema multiforme affect oral mucosa
buccal mucosa, tongue, labial mucosa most common
67
etiology of erythema multiforme
Herpes simplex infection
Drug reaction: Sulfonamides, Penicillins, Quinolones, Chlormezanone, Barbiturates, NSAIDS, Anticonvulsants, Allopurinol
68
immunofluorescence of erythema multiforme
Negative
Rules out other vesiculobullous diseases
69
tx if erythema multiforme
Antihistamines
Topical anesthetic, debridement of lesions
Some use of steroids
70
what are drug eruptions
certain medications may cause oral lesions resembling lichen planus or vesiculobullous disease
71
what are drug eruption diseases
Lichenoid Drug Eruptions
Lichenoid Mucositis
72
what can cause drug eruptions
1. medications
2. cinnamon
3. cinnamic aldehyde
73
what is used to mask taste of pyrophosphates in tartar control toothpase
cinnamic aldehyde
74
drugs associated with lichenoid drug eruptions
75
tx of drug eruptions
discontinue offending agent
76
what are differential diagnoses
Lichen Planus
Cicatricial Pemphigoid
Pemphigus Vulgaris
Linear IgA Disease
Lupus Erythematosus
Erythema Multiforme
Drug Eruptions
