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Dental Management of a Complex Patient > Autoimmune & Inflammatory Disorders > Flashcards

Autoimmune & Inflammatory Disorders Flashcards

1
Q

Autoimmune and Connective Tissue Diseases
(4)

A
  1. Systemic Lupus Erythematosus
  2. Rheumatoid Arthritis
  3. Progressive Systemic Sclerosis
  4. Sjögren’s Syndrome
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2
Q

skipped
Immune-mediated Conditions
(6)

A
  1. Contact Stomatitis
  2. Angioedema
  3. Orofacial Granulomatosis
  4. Sarcoidosis
  5. Erythema Multiforme/Stevens–Johnson Syndrome/Toxic Epidermal Necrolysis
  6. Lichen Planus
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3
Q

Vesiculobullous Conditions
(4)

A
  1. Pemphigus Vulgaris
  2. Bullous Pemphigoid
  3. Mucous Membrane Pemphigoid
  4. Paraneoplastic Pemphigus
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4
Q

Autoimmunity Definition

A
  • presence of antibodies (auto-antibodies or auto-reactive Tcells) directed against normal host antigens (auto- or selfantigens)
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5
Q

Systemic Lupus Erythematosus
Pathogenesis
(2)

A
  • Unknown etiology
  • Chronic, inflammatory autoimmune
    disorder
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6
Q
  • Unknown etiology
    (1)
A
  • Environmental triggers in a
    genetically predisposed individual
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7
Q
  • Chronic, inflammatory autoimmune
    disorder
    (2)
A
  • Autoantibodies and immune
    complexes activate complement
    system
  • Vasculitis, fibrosis, tissue necrosis
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8
Q

Systemic Lupus Erythematosus
Complications

A

Multi-organ

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9
Q

Systemic Lupus Erythematosus
Epidemiology
(2)

A
  • 90% are young-middle aged women
  • 2.5 times increased risk in AA
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10
Q

Systemic Lupus Erythematosus
Diagnosis
American College of Rheumatology
criteria for the diagnosis of SLE requires 4
of the 11 criteria to be met:

A
  1. Arthritis
  2. Serositis (pleuritis or pericarditis)
  3. Malar rash
  4. Discoid rash
  5. Photosensitivity
  6. Oral ulcers
  7. Renal disease
  8. Neurological disease (psychosis
    or seizures)
  9. Hematological disease (hemolytic
    anemia, thrombocytopenia,
    leukopenia, or lymphopenia)
  10. Immunological manifestations
    (anti-DS DNA, anti-SM,
    antiphospholipid antibodies)
  11. Antinuclear antibodies
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11
Q

Systemic Lupus Erythematosus
Management
Rheumatologists:
(2)

A

organ-specific approach
* Long-term Prednisone
* Immunomodulating agents (eg.
mycophenolate mofetil, azathioprine,
methotrexate)

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12
Q

Systemic Lupus Erythematosus
Oral Manifestations
SLE-like lichenoid lesions
(3)

A
  • Ulcerations/erosions
  • Hard palatal mucosa ulcer
  • White radiating striae from a central
    ulcer
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13
Q

Systemic Lupus Erythematosus
Dental Considerations
Determine the status of the stability of
their disease
(5)

A

Leukopenia
Thrombocytopenia (25% of pts)
Nonbacterial verrucous valvular Libman–
Sacks endocarditis
Renal disease
Neuropsychiatric disease

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14
Q

Leukopenia

A
  • Immunosuppressive rx
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15
Q

Thrombocytopenia (25% of pts)

A
  • extreme thrombocytopenia
    (<20,000 platelets)
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16
Q

Nonbacterial verrucous valvular Libman–
Sacks endocarditis

A
  • most common cardiac lesion
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17
Q

Renal disease
(2)

A
  • localization of immune complexes
    in the kidney is the precipitating
    factor in the development of
    lupus nephritis
  • rapidly progressing
    glomerulonephritis or a less
    aggressive form
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18
Q

Neuropsychiatric disease

A
  • psychosis, seizures,
    cerebrovascular accidents
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19
Q

Rheumatoid Arthritis
Pathogenesis
Unknown etiology
* Environmental, hormonal,
infectious factors, in a genetically
predisposed individual
(3)

A
  • external trigger (infection,
    trauma) elicits an
    autoimmune reaction
  • hypertrophy of the synovial
    lining of the joint and
    endothelial cell activation
  • uncontrolled inflammation
    and destruction of cartilage
    and bone
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20
Q

Rheumatoid Arthritis
Complications

A

Joints and extra-articular involvement

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21
Q

Rheumatoid Arthritis
Epidemiology
(3)

A

1.3 million US adults
* Women 3 times more affected than
men
* sex difference diminish in older
age groups, suggesting a
hormonal component

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22
Q

Rheumatoid Arthritis
Diagnosis
Diagnosis 6/10 score based on a 4-prong algorithm:
(4)

A
  1. joint involvement
  2. serology test results (Rheumatoid
    factor, ACPA anticitrullinated protein
    antibody)
  3. acute-phase reactant test results (CRP,
    ESR)
  4. pt self-reporting of signs/symptom
    duration
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23
Q

RA
Management
Nonpharmacological:

Pharmacological:

A
  • Physical, occupational therapies,
    orthotic devices, surgery
  • NSAIDs, disease-modifying
    antirheumatic drugs,
    immunosuppressants, biological
    response modifiers, corticosteroids
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24
Q

RA
Oral Manifestations
(3)

A
  • TMJ dysfunction
  • Medication induced ulcerations
  • Salivary hypofunction
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25
Q

RA
Dental Considerations

A

See ADA guideline on prophylactic
antibiotics for pts with prosthetic joints
undergoing dental procedures

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26
Q

Progressive Systemic Sclerosis
(Scleroderma)
Pathogenesis
(3)

A

Unknown etiology
* Endothelial cell injury
* Fibroblast activation
* Vascular dysfunction

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27
Q

Progressive Systemic Sclerosis
(Scleroderma)
Complications
(2)

A
  • skin thickening/induration
  • prominent fibroproliferative vascular
    disease
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28
Q
  • skin thickening/induration
    (2)
A
  • tissue fibrosis and chronic
    inflammation
  • infiltration of heart, lungs and
    kidneys
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29
Q

Progressive Systemic Sclerosis
(Scleroderma)
Epidemiology
(2)

A
  • Peak onset (30–50 years)
  • 4-9 times higher in women than in
    men
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30
Q

Progressive Systemic Sclerosis
(Scleroderma)
Diagnosis
Localized scleroderma:
(3)

A
  • skin on the hands and face
  • slow disease course
  • indolent and rarely spreads more
    widely or results in serious
    complications
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31
Q

Progressive Systemic Sclerosis
(Scleroderma)
Diagnosis
Systemic scleroderma:
(1)

A
  • affects large areas of skin and heart,
    lungs, or kidneys
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32
Q

2 main types of systemic scleroderma:
(2)

A

(1) limited disease or CREST syndrome,
calcinosis, Raynaud’s syndrome,
esophageal dysmotility, sclerodactyly,
telangiectasia
(2) diffuse disease

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33
Q

Progressive Systemic Sclerosis
(Scleroderma)
Management
(2)

A
  • Long-term Prednisone
  • Immunomodulating agents (eg.
    mycophenolate mofetil, azathioprine,
    methotrexate)
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34
Q

Progressive Systemic Sclerosis
(Scleroderma)
Oral Manifestations

A

Severe microstomia, trismus, submucosal
fibrosis

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35
Q

Progressive Systemic Sclerosis
(Scleroderma)
Dental Considerations

A

Elongation exercises to improve trismus

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36
Q

Sjogrens Syndrome
Pathogenesis
(3)

A
  • Chronic, autoimmune, inflammatory
    disorder
  • Primary vs Secondary SS (RA, SLE)
  • Unknown etiology
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37
Q

Sjogrens Syndrome
* Unknown etiology
(3)

A
  • expression of MHC-II molecules in
    activated salivary gland cells
  • inherited susceptibility markers
    trigger a chronic inflammatory
    response in genetically
    susceptible individuals
  • ongoing activation of the innate
    immune system as proinflammatory cytokines (eg. IFN-g)
    are elevated
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38
Q

Sjogrens Syndrome
Complications
(3)

A
  • Lymphocytic infiltration
  • lacrimal glands causing dry eyes
    (xerophthalmia)
  • salivary glands causing drymouth
    (xerostomia)
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39
Q

Sjogrens Syndrome
Epidemiology
(2)

A
  • Elderly women
  • Female-to-male ratio is 9 : 1
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40
Q

Sjogrens Syndrome
Diagnosis
SS ACR Classification Crietria (2/3)
1. Positive
2. Minor labial salivary gland biopsy
exhibiting focal lymphocytic
sialadenitis focus score:
3. Ocular staining score:

A

anti-Ro and/or anti-La or (+RF and ANA titer >1:320)
>1 focus/4mm2
>3

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41
Q

Sjogrens Syndrome
Management
(3)

A
  • Rituximab (anti-CD20 monoclonal
    antibodies)
  • Long-term Prednisone
  • Immunomodulating agents (eg.
    mycophenolate mofetil, azathioprine,
    methotrexate)
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42
Q

Sjogrens Syndrome
Dental Considerations
(3)

A

Salivary hypofunction
* stimulated and unstimulated
salivary flow measurements
* 44 times increased risk of MALToma

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43
Q

Melkerson-Rosenthal
Syndrome
(3)

A
  • Lip swelling
  • Fissured tongue
  • Facial paralysis
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44
Q

OFG vs. oral Crohn disease
* Evaluation of 207 patients
* —% of new cases developed/had CD (46pts)
* median interval between the diagnoses was 2 years
* –% had Melkerson-Rosenthal Syndrome (5pts)

A

22
2

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45
Q

Probability of CD
Over –% of children dx with OFG will have CD in 10 years and
–% are already dx

A

22
8

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46
Q

Sites affected in 207 patients
* Mostly swelling of —
* –% had swelling and –% had cobblestoning on BM

A

lips
57, 27

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47
Q

Gingival involvement
Ranges from

A

segmental or diffuse erythema to edematous
hyperplastic gingiva +/- pain

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48
Q

Angular cheilitis
28% of which 61% had — infection

A

Staph. aureus

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49
Q

Characteristics of 35 OFG
patients
* Median age – years
* Cobblestoning –%
* Fissuring –%
* Aphthous-like ulceration –%
* Deeper linear-type ulcers –%

A

24
49
37
15
12

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50
Q

Comprehensive literature review
on food sensitivity
(7)

A
  • Benzoic acid (36%)
  • Food additives (33%)
  • Perfumes and flavourings (28%)
  • Cinnamaldehyde (27%)
  • Cinnamon (17%)
  • Benzoates (17%)
  • Chocolate (11%)
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51
Q

Cinnamon and benzoate free diet
–% had benefit
–% need no other therapy

A

54-78
25

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52
Q

Treatment
(3)

A
  • Topical/intralesional/systemic corticosteroids
  • Various response
  • Adjuvant therapies
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53
Q
  • Topical/intralesional/systemic corticosteroids
A
  • delayed release triamcinolone
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54
Q

skipped
* Adjuvant therapies

A
  • clofazimine, sulfasalazine, hydroxychloroquine, methotrexate,
    danazol, dapsone, TNF-alpha ant., metronidazole
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55
Q

Sarcoidosis
* Multisystem
diseases involves
the (3)
* — radiographic
changes during a
routine screening
examination
* Systemic
symptoms: (3)

A

lungs, eyes, and skin
Chest
fatigue,
night sweats, and weight loss

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56
Q

Sarcoidosis
Pathogenesis
(3)

A
  • Development and accumulation of granulomas (Compact,
    centrally organized collections of macrophages and
    epithelioid cells encircled by lymphocytes)
  • Granulomas generally form to confine pathogens, restrict
    inflammation, and protect surrounding tissue
  • Macrophages, with chronic cytokine stimulation,
    differentiate into epithelioid cells, gain secretory and
    bactericidal capability, lose some phagocytic capacity, and
    fuse to form multinucleated giant cells
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57
Q

Lofgren’s syndrome
* — presentation
*(3)
* Erythema nodosum is observed
predominantly in —
* Marked ankle periarticular
inflammation or arthritis without
erythema nodosum is more
common in —

A

Acute
Arthritis, erythema nodosum, and
bilateral hilar adenopathy (9 to 34%
of patients)
women
men

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58
Q

Lofgren’s syndrome
Oral Presentation
(2)

A
  • Plaque or nodular lesion
  • Tongue and lip
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59
Q

Lofgren’s syndrome
Treatment
(4)

A
  • Oral prednisone (20 to 40 mg/day); international expert panel
  • Evaluate the response after 1 to 3 months
  • If there has been a response, the prednisone dose should be
    tapered to 5 to 15 mg per day, with treatment planned for an
    additional 9 to 12 months
  • Lack of a response after 3 months suggests the presence of
    irreversible fibrotic disease, or an inadequate dose of
    prednisone
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60
Q

Lofgren’s syndrome
Transplantation

A
  • About 3% of lung transplantations and less than 1% of heart
    and liver transplantations are performed in patients with
    sarcoidosis
61
Q

Lofgren’s syndrome
Prognosis
* — of patients with sarcoidosis generally have a remission
within a decade after diagnosis
* A recurrence after — of remission is uncommon
(affecting <5% of patients)
* Less than —% of patients die from sarcoidosis
* Death as a result of (2)

A

2/3
1 or more years
5
pulmonary fibrosis with respiratory failure
or of cardiac or neurologic involvement

62
Q

Erythema multiforme
(4)

A
  • Acute hypersensitivity reaction to various antigens
  • Characterized clinically by target lesions distributed symmetrically
    on extremities and trunk
  • Usually self-limiting but may be recurrent
  • Many different classifications exist
63
Q

Erythema multiforme
Epidemiology
* Prevalence –
* Young adults —
* — M:F ratio
* Reported recurrence rate: —%
* Oral involvement: as high as –%

A

< 1%
20-40 years old
1:1.5
37
70

64
Q

Erythema multiforme
Clinical presentation
(3)

A
  • Vesiculobullous condition that may affect skin or mucous
    membranes
  • Symmetric involvement of extremities and trunk
  • Characteristic lesion: target/iris lesion
65
Q

Erythema multiforme
‘Multiform’ clinical features:
(4)

A
  • Characteristic target lesions
  • Macules
  • Vesicles
  • Papules
66
Q

Erythema multiforme
Clinical presentation
* HAEM (Herpes associated EM):
(2)
* DIEM (drug induced EM):
(2)

A
  • No or mild prodromal symptoms
  • More likely to be recurrent
  • Flu-like prodrome common
  • Less likely to be recurrent
67
Q

Etiology and pathogenesisHAEM
(4)

A
  • 70-80%: HSV infection is precipitating event
  • HSV-specific CD4+ Th1 cells and inflammatory cytokines (IFN-γ)
    recruit auto-reactive CD8+ T-cells
  • CLA+ (skin-homing) CD8+ T-cells triggered by viral antigen-positive
    cells
  • Mechanism of auto-reactive T-cell generation unclear
68
Q
  • CLA+ (skin-homing) CD8+ T-cells triggered by viral antigen-positive
    cells
    (3)
A
  • Keratinocyte growth arrest
  • Keratinocyte lysis and apoptosis
  • Release of cytotoxic factors
69
Q

Variations in classification
* EM minor:
(2)
* EM major:
(2)

A
  • Rashes/target lesions symmetrically distributed on extremities
  • Mucous membrane involvement limited to one surface ->
    typically oral mucosa
  • Skin lesions may also be atypical -> raised, bullous
  • Involvement of multiple mucous membranes
70
Q
  • Classic EM:
A
  • Acute onset, self-limiting, lasts 2-4 weeks
71
Q
  • Recurrent EM:
A
  • More likely to be Herpes-associated
72
Q
  • Persistent EM: rare
    (3)
A
  • Continuous eruption of typical and atypical lesions
  • Often widespread and necrotic
  • May be due to underlying viral infection, inflammatory condition,
    malignancy, or idiopathic
73
Q

EM
Management
* Treatment of (2)
* Complete recovery usually occurs in — weeks
* No treatment identified that predictably alters —
* Topical or systemic —
* 0.5-1.0 mg/kg/day tapered over 7-10days
* Steroid — agents
* Lesions usually heal without —

A

relevant infections or removal of causal drugs
2-4
clinical course
steroids
sparing
scarring

74
Q

Stevens-Johnson syndrome
*
* Etiology, genetic susceptibility, and pathomechanism distinct from
—
* Etiology, genetic susceptibility, and pathomechanism identical to
—
* Now thought to form single disease entity, distinct from EM:

A

Severe cutaneous blistering hypersensitivity reaction clinically
similar to EM but with more frequent mucosal involvement
EM
Toxic epidermal necrolysis
SJS/TEN

75
Q

Stevens-Johnson syndrome/Toxic
epidermal necrolysis
* Severe cutaneous adverse reaction that affects skin and
mucosal surfaces
(3)

A
  • Erythema and blister formation of varying extent
  • Hemorrhagic erosions of mucous membranes
  • Fever and malaise:
  • Often first sign of disease
  • May persist after mucocutaneous lesions appear
76
Q

Stevens-Johnson syndrome/Toxic
epidermal necrolysis
* Mainly caused by

A

drugs but infections and other unidentified
risk factors may play a role

77
Q

Stevens-Johnson syndrome/Toxic
epidermal necrolysis
Pathophysiology
* 75% of SJS/TEN cases attributable to —
* Median latency time: —weeks
* 74-94% of TEN cases attributable to —
* —, mediated by cytokines, likely play an important
role in epidermal necrosis
* Underlying mechanism still unknown

A

medications
< 4
medications or URT infection
CD8+ T-cells

78
Q

Stevens-Johnson syndrome/Toxic
epidermal necrolysis
Prognosis
* Prognosis depends on degree of — involvement
* SJS: –% mortality rate
* SJS/TEN overlap: –% mortality rate
* TEN: up to –% mortality rate
* — is most common cause of death

A

epidermal
10
30
50
Infection

79
Q

Oral Lichen Planus (OLP)
*
* Stratified squamous epithelium of (4)
* Is it an auto-immune condition?
* No circulating —
* Etiology
* –% adult population
* Most common skin condition with — involvement
* Age: —
* Gender

A

T-cell immune mediated chronic muco-cutaneous inflammatory
disease
skin, oral mucosa, genital mucosa, larynx
auto-antibodies
Unknown
1-2
oral
30-60yrs
F>M (1.4:1)

80
Q

OLP
Predisposing Factors
(3)

A
  • Lichenoid hypersensitivity reaction
  • Viral infections
  • Diabetes Mellitus
81
Q

OLP
* Lichenoid hypersensitivity reaction
* Clinically & histologically indistinguishable from LP
(7)

A
  • Anti-hypertensives
  • NSAIDs
  • Statins
  • Anti-diabetic agents
  • Amalgam restorations
  • Cinnamon flavouring agents
  • Anti-hypothyroidism agents
82
Q

OLP
* Viral infections
(2)

A
  • HPV
  • Hep C
83
Q

OLP
Clinical Features
(3)

A
  • 10-15% have skin LP
  • 6 P’s
  • Koebner phenomenon
84
Q

OLP
* 6 P’s

A
  • Polygonal
  • Pruritic
  • Purple
  • Papular
  • Planar
  • Plaques
85
Q

OLP
* Koebner phenomenon
(3)

A
  • Wickham striae on flexor
    surface of wrists
  • LP lesions at sites of trauma
  • Does Koebnerization occur
    in the oral cavity?
86
Q

OLP
Clinical Features
* Reticular Form
(4)

A
  • Classic wickham striae
  • Bilateral BM
  • Attached gingiva
  • Ventral/dorsal tongue
87
Q

OLP
* Erosive/Ulcerative
form
(3)

A
  • Most painful
  • Yellow fibrin membrane
  • Higher rate of malignant
    transformation
88
Q

Desquamative Gingivitis
Histopathology
(5)

A
  • Hyperkeratosis
  • Chronic dense band- like infiltrate of
    lymphocytes
  • Saw tooth epithelial
    rete pegs/ridges
  • Degeneration of basal
    cell layer
  • Colloid/Civette bodies
89
Q

Desquamative Gingivitis
Hyperkeratosis
(2)

A
  • Orthokeratosis
  • Parakeratosis
90
Q

Desquamative Gingivitis
* Colloid/Civette bodies

A
  • degenerating
    keratinocytes
91
Q

Desquamative Gingivitis
Direct Immunofluorescence

A
  • Shaggy/patchy/granu
    lar deposits of C3
    fibrinogen at BMZ
92
Q

Desquamative Gingivitis
Management
* Cutaneous lesions

A
  • Refer to dermatology
93
Q

skipped
Desquamative Gingivitis
Management
* Topical
(7)

A
  • Fluocinonide gel 0.05% BID-QID
  • Clobetasol gel 0.05% BID-QID
  • Kenalog 40 mg (triamcinolone injection) 10 mg per 1cm ulcer (0.25 mls)
  • Dexamethasone 0.5mg/5ml BID-QID 5ml swish & spit 5mins
  • Clobetasol solution 0.05% BID-QID 5ml swish & spit 5mins
  • Tacrolimus 0.1% BID-QID 5ml swish & spit 5mins
  • Tacrolimus & Clobetasol combined rinses
94
Q

Desquamative Gingivitis
Management
* Systemic
(2)

A
  • Prednisone 1mg/kg (5-7days)
  • Plaquenil (hydroxychloroquine) 200 mg BID
95
Q

Desquamative Gingivitis
Malignant Potential
* Mechanisms
(3)

A
  • Chronic inflammation
  • Immune dysregulation
  • Topical/Systemic
    immunosuppressants
96
Q

Pemphigus Vulgaris (PV)
*
* Incidence: — cases per year
* Median Age:
* Gender
* Mediterranean, South Asian or Jewish heritage
* (3) associated with PV

A

Autoimmune vesicullobullous condition
5/1,000,000
50 yrs
No sex predilection
HLA-DR4, DRw14 & DQB1

96
Q

Desquamative Gingivitis
* Risk (0.1-0.2%)
(6)

A
  • Very low, 1/500-1000 OLP
    pts develop OSCC
  • Yet to be confirmed
  • To date poorly
    documented
  • Erosive/ulcerative LP
  • Smokers & Alcohol
  • Monitor LP annually
97
Q
  • Autoimmune vesicullobullous condition
    (4)
A
  • Pemphigus subtypes
  • pemphigus vulgaris
  • pemphigus vegetans
  • pemphigus erythematosus
  • pemphigus foliaceus
98
Q

PV
Clinical Features
(2)

A
  • Nikolsky sign
  • application of firm
    lateral pressure on
    normal-appearing
    mucosa adjacent to a
    pre-existing bulla
    induces new bulla
    formation
99
Q

PV
Clinical Features
* Asboe-Hansen sign

A
  • application of pressure
    directly to a bulla causes
    lateral extension
100
Q

PV
Oral Features
* “first to show, last to
go”
(3)

A
  • oral lesions 1st sign >50%
    of cases >1 year
  • almost all have oral
    features
  • most difficult to resolve
    with tx
101
Q

PV
Oral Features
* Untreated oral &
cutaneous lesions
(1)

A
  • persist progressively
    involving more surfaces
102
Q

PV
Histopathology
(3)

A
  • Intraepithelial
    separation
  • Acantholysis
  • Lamina propria
103
Q

PV
* Acantholysis
(2)

A
  • epithelial spinous layer
    cells fall apart
  • rounded shape for loose
    cells (Tzanck cells)
104
Q

PV
* Lamina propria
(1)

A
  • mild-moderate chronic
    inflammatory cell
    infiltrate
105
Q

PV
Immunofluorescence Studies
* DIF
(1)
* InDIF & ELISA
(2)

A
  • Intercellular IgG/IgM ± C3
  • +ve in 80-90% cases
  • circulating autoantibodies
106
Q

PV
Management
(5)

A
  • Better prognosis/control if dx early
  • Prednisone
  • Steroid-sparing agents
  • Monitor circulating autoantibody titres via inDIF to gauge success of tx
  • 75% pts have disease resolution 10 yrs s/p tx
107
Q

PV
* Better prognosis/control if dx early

A
  • before dev of corticosteroid tx 60-90% died due to dehydration, electrolyte
    imbalances, malnutrition & infection
108
Q

PV
* Prednisone
(2)

A
  • 5-10% mortality rate now due to complications of long-term corticosteroid
    use
  • alternate day prednisone plus steroid-sparing immunosuppressant
109
Q

PV
* Steroid-sparing agents
(7)

A
  • azathioprine
  • dapsone
  • mycophenolate mofetil
  • cyclophosphamide
  • IVIg
  • plasmapheresis
  • rituximab
110
Q

Bullous Pemphigoid (BP)
*
- 1 in — annually
* Age:
* Gender:
* Oral involvement

A

Most common autoimmune blistering condition
100,000
6th-8th decade
2:1 male
uncommon, 8-39%

111
Q

BP
Histopathology
* Perilesional biopsy
(3)

A
  • subepithelial
    separation
  • eosinophils within
    bulla
  • acute & chronic
    inflammatory cells
112
Q

BP
Direct Immunofluorescence
* BMZ
(1)
* Hemidesmosomes
(2)

A
  • linear IgG & C3 (90-
    100%)
  • BP180 (lamina lucida)
  • BP230
113
Q

BP
Indirect Immunofluorescence
* Antibody titers
(2)

A
  • 50-90% +ve circulating
    autoantibodies
  • no correlation with disease
    activity
114
Q

BP
Binding of
autoantibodies to BMZ
(4)

A
  • starts the complement
    cascade
  • degranulation of mast cells
  • eosinophils & neutrophils
    recruitment
  • elastases & matrix
    metalloproteinases
115
Q

BP
Management
* Good prognosis
(2)

A
  • spontaneous remission after 2-5 years
  • up to 27% mortality rate
116
Q

BP
* Systemic immunosuppressive therapy
(2)

A
  • Prednisone QD/QOD
  • Azathioprine added if no response
117
Q

BP
* Alternative therapies
(3)

A
  • Dapsone
  • Tetracycline
  • Niacinamide
118
Q

BP
* Refractory cases
(1)

A
  • Prednisone & Cyclophosphamide
119
Q

Mucous Membrane Pemphigoid
(MMP)
*
* Autoantibodies to —
* Positive — sign
* Unknown incidence
- 2x as common as –
- most common –
* Age:
* Gender:

A

Chronic, blistering, mucocutaneous autoimmune disease
hemidesmosomes
Nikolsky
PV
oral AD
5th-6th decade
2:1 female

120
Q

Sub-types of MMP
(4)

A
  • Ocular involvement only
  • Oral involvement only
  • Mucosal & cutaneous involvement
  • Multiple mucosal sites without cutaneous involvement
  • nose, esophagus, larynx, vagina
121
Q

BP
Ocular Complications
* Conjunctival mucosal
scarring
(3)

A
  • Entropion
  • Symblepahron
  • Trichiasis
122
Q

BP
Ocular Complications
* Scarring closes
lacrimal gland
openings
(3)
* Blindness
(2)

A
  • loss of tears
  • extremely dry
  • cornea produces
    excess keratin
  • if untreated
  • opacification from
    excess keratinization
123
Q

BP
Histopathology
* Perilesional biopsy
(3)

A
  • BM separation
  • subepithelial clefting
  • inflammatory infiltrate
    superficial lamina
    propria
124
Q

BP
Direct Immunofluorescence
* Perilesional biopsy
(2)

A
  • linear band IgG & C3
    (90%)
  • IgA & IgM (more
    severe)
125
Q

BP
Indirect Immunofluorescence
(3)

A
  • MMP lacks detectable circulating autoantibody levels
  • Anti-epiligrin MMP
  • Oral MMP only
126
Q

Indirect Immunofluorescence
BP
* MMP lacks detectable circulating autoantibody levels
(1)

A
  • 5-25% pts. +ve
127
Q

Indirect Immunofluorescence
BP
* Anti-epiligrin MMP
(2)

A
  • autoantibodies to epiligrin (laminin-5)
  • more widespread involvement
128
Q

Indirect Immunofluorescence
BP
* Oral MMP only
(1)

A
  • autoantibodies to a6 integrin
129
Q

BP
Management
(3)

A
  • Referral to an ophthalmologist
  • Referral to a dermatologist
  • Topical corticosteroids (custom trays)
130
Q
  • Referral to an ophthalmologist
  • –% of oral MMP pts develop ocular lesions
A

25

131
Q
  • Referral to a dermatologist
  • –% of oral MMP pts develop cutaneous lesions
A

20

132
Q
  • Topical corticosteroids (custom trays)
    (2)
A
  • 0.05% fluocinonide gel BID
  • 0.05% clobetasol gel BID
133
Q

Paraneoplastic Pemphigus
(PNP)
(3)

A
  • Neoplasia-induced Pemphigus
  • Paraneoplastic Autoimmune Multi-organ Syndrome (PAMS)
  • Rare vesicullobullous disorder
  • 150 documented cases
134
Q

Paraneoplastic Pemphigus
(PNP)
* Neoplasm hx
(5)

A
  • Non-Hodgkin Lymphoma (42%)
  • Chronic Lymphocytic Leukemia (29%)
  • Sarcoma (6%)
  • Thymoma (6%)
  • Castleman Disease (6%)
135
Q

PNP
Pathogenesis
(5)

A
  • Unknown
  • Multifaceted immunologic attack
  • Evidence suggests abnormal cytokine levels
  • IL-6 produced by lymphocytes in response to patient’s tumor
  • Mediated by cytotoxic T-lymphocytes in some cases
136
Q
  • IL-6 produced by lymphocytes in response to patient’s tumor
    (2)
A
  • IL-6 stimulates abnormal production of antibodies
  • antibodies against desmosomal complex antigens
137
Q

PNP
Clinical Features
* PNP developed prior to malignancy dx

A
  • 1/3 of reported cases
138
Q

PNP
* Signs & Symptoms
(2)

A
  • sudden appearance
  • polymorphous
139
Q

PNP
* Cutaneous lesions
(3)

A
  • bullaeerosionstarget lesionslichenoid lesions
    erythematous papules
  • papular & pruritic ( cutaneous LP)
  • palmar or plantar bullae (uncommon in PV)
140
Q

PNP
* found where

A

Vagina, respiratory tract, oral & conjunctival mucosa
(scarring similar to MMP)

141
Q

PNP
Oral Manifestations
“Erythema multiformelike stomatitis”
(4)

A
  • Hemorrhagic lip
    crusting
  • All sites
  • Oral involvement
    only in some cases
  • Multiple painful
    irregular ulcers
142
Q

PNP
Histopathology
* Non-specific
(3)

A
  • lichenoid mucositis
  • intraepithelial clefting
  • subepithelial clefting
143
Q

PNP
Direct Immunofluorescence
(2)

A
  • Weakly +ve linear
    IgG/C3 along
    intracellularly & BMZ
  • InDIF
144
Q

PNP
InDIF
(5)

A
  • desmoplakin I & II
  • major bullous
    pemphigoid antigen
  • envoplakin
  • periplakin
  • desmoglein 1 & 3
145
Q

PNP
Immunoblotting
(2)

A
  • Considered the gold
    standard for PNP
    diagnosis
  • Characteristic
    reactivity with two
    plakin proteins is
    highly sensitive and
    specific for PNP
146
Q

PNP
Management
(2)

A
  • High morbidity & mortality (>90%)
  • Systemic prednisone + immunosuppressant
147
Q

PNP
* High morbidity & mortality (>90%)
* complications from — lesions
* — therapy
* trigger a reactivation of —
* ~ –% develop bronchiolitis obliterans

A

vesiculobullous
immunosuppressive
malignant neoplasm
50

148
Q

PNP
* Systemic prednisone + immunosuppressant
(3)

A
  • azathioprine
  • methotrexate
  • cyclophosphamide

Dental Management of a Complex Patient (13 decks)

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