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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
RA Dental Considerations
See ADA guideline on prophylactic antibiotics for pts with prosthetic joints undergoing dental procedures
26
Progressive Systemic Sclerosis (Scleroderma) Pathogenesis (3)
Unknown etiology * Endothelial cell injury * Fibroblast activation * Vascular dysfunction
27
Progressive Systemic Sclerosis (Scleroderma) Complications (2)
* skin thickening/induration * prominent fibroproliferative vascular disease
28
* skin thickening/induration (2)
* tissue fibrosis and chronic inflammation * infiltration of heart, lungs and kidneys
29
Progressive Systemic Sclerosis (Scleroderma) Epidemiology (2)
* Peak onset (30–50 years) * 4-9 times higher in women than in men
30
Progressive Systemic Sclerosis (Scleroderma) Diagnosis Localized scleroderma: (3)
* skin on the hands and face * slow disease course * indolent and rarely spreads more widely or results in serious complications
31
Progressive Systemic Sclerosis (Scleroderma) Diagnosis Systemic scleroderma: (1)
* affects large areas of skin and heart, lungs, or kidneys
32
2 main types of systemic scleroderma: (2)
(1) limited disease or CREST syndrome, calcinosis, Raynaud’s syndrome, esophageal dysmotility, sclerodactyly, telangiectasia (2) diffuse disease
33
Progressive Systemic Sclerosis (Scleroderma) Management (2)
* Long-term Prednisone * Immunomodulating agents (eg. mycophenolate mofetil, azathioprine, methotrexate)
34
Progressive Systemic Sclerosis (Scleroderma) Oral Manifestations
Severe microstomia, trismus, submucosal fibrosis
35
Progressive Systemic Sclerosis (Scleroderma) Dental Considerations
Elongation exercises to improve trismus
36
Sjogrens Syndrome Pathogenesis (3)
* Chronic, autoimmune, inflammatory disorder * Primary vs Secondary SS (RA, SLE) * Unknown etiology
37
Sjogrens Syndrome * Unknown etiology (3)
* 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
38
Sjogrens Syndrome Complications (3)
* Lymphocytic infiltration * lacrimal glands causing dry eyes (xerophthalmia) * salivary glands causing drymouth (xerostomia)
39
Sjogrens Syndrome Epidemiology (2)
* Elderly women * Female-to-male ratio is 9 : 1
40
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:
anti-Ro and/or anti-La or (+RF and ANA titer >1:320) >1 focus/4mm2 >3
41
Sjogrens Syndrome Management (3)
* Rituximab (anti-CD20 monoclonal antibodies) * Long-term Prednisone * Immunomodulating agents (eg. mycophenolate mofetil, azathioprine, methotrexate)
42
Sjogrens Syndrome Dental Considerations (3)
Salivary hypofunction * stimulated and unstimulated salivary flow measurements * 44 times increased risk of MALToma
43
Melkerson-Rosenthal Syndrome (3)
* Lip swelling * Fissured tongue * Facial paralysis
44
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)
22 2
45
Probability of CD Over --% of children dx with OFG will have CD in 10 years and --% are already dx
22 8
46
Sites affected in 207 patients * Mostly swelling of --- * --% had swelling and --% had cobblestoning on BM
lips 57, 27
47
Gingival involvement Ranges from
segmental or diffuse erythema to edematous hyperplastic gingiva +/- pain
48
Angular cheilitis 28% of which 61% had --- infection
Staph. aureus
49
Characteristics of 35 OFG patients * Median age -- years * Cobblestoning --% * Fissuring --% * Aphthous-like ulceration --% * Deeper linear-type ulcers --%
24 49 37 15 12
50
Comprehensive literature review on food sensitivity (7)
* Benzoic acid (36%) * Food additives (33%) * Perfumes and flavourings (28%) * Cinnamaldehyde (27%) * Cinnamon (17%) * Benzoates (17%) * Chocolate (11%)
51
Cinnamon and benzoate free diet --% had benefit --% need no other therapy
54-78 25
52
Treatment (3)
* Topical/intralesional/systemic corticosteroids * Various response * Adjuvant therapies
53
* Topical/intralesional/systemic corticosteroids
* delayed release triamcinolone
54
skipped * Adjuvant therapies
* clofazimine, sulfasalazine, hydroxychloroquine, methotrexate, danazol, dapsone, TNF-alpha ant., metronidazole
55
Sarcoidosis * Multisystem diseases involves the (3) * --- radiographic changes during a routine screening examination * Systemic symptoms: (3)
lungs, eyes, and skin Chest fatigue, night sweats, and weight loss
56
Sarcoidosis Pathogenesis (3)
* 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
57
Lofgren’s syndrome * --- presentation *(3) * Erythema nodosum is observed predominantly in --- * Marked ankle periarticular inflammation or arthritis without erythema nodosum is more common in ---
Acute Arthritis, erythema nodosum, and bilateral hilar adenopathy (9 to 34% of patients) women men
58
Lofgren’s syndrome Oral Presentation (2)
* Plaque or nodular lesion * Tongue and lip
59
Lofgren’s syndrome Treatment (4)
* 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
60
Lofgren’s syndrome Transplantation
* About 3% of lung transplantations and less than 1% of heart and liver transplantations are performed in patients with sarcoidosis
61
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)
2/3 1 or more years 5 pulmonary fibrosis with respiratory failure or of cardiac or neurologic involvement
62
Erythema multiforme (4)
* 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
Erythema multiforme Epidemiology * Prevalence -- * Young adults --- * --- M:F ratio * Reported recurrence rate: ---% * Oral involvement: as high as --%
< 1% 20-40 years old 1:1.5 37 70
64
Erythema multiforme Clinical presentation (3)
* Vesiculobullous condition that may affect skin or mucous membranes * Symmetric involvement of extremities and trunk * Characteristic lesion: target/iris lesion
65
Erythema multiforme ‘Multiform’ clinical features: (4)
* Characteristic target lesions * Macules * Vesicles * Papules
66
Erythema multiforme Clinical presentation * HAEM (Herpes associated EM): (2) * DIEM (drug induced EM): (2)
* No or mild prodromal symptoms * More likely to be recurrent * Flu-like prodrome common * Less likely to be recurrent
67
Etiology and pathogenesisHAEM (4)
* 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
* CLA+ (skin-homing) CD8+ T-cells triggered by viral antigen-positive cells (3)
* Keratinocyte growth arrest * Keratinocyte lysis and apoptosis * Release of cytotoxic factors
69
Variations in classification * EM minor: (2) * EM major: (2)
* 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
* Classic EM:
* Acute onset, self-limiting, lasts 2-4 weeks
71
* Recurrent EM:
* More likely to be Herpes-associated
72
* Persistent EM: rare (3)
* Continuous eruption of typical and atypical lesions * Often widespread and necrotic * May be due to underlying viral infection, inflammatory condition, malignancy, or idiopathic
73
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 ---
relevant infections or removal of causal drugs 2-4 clinical course steroids sparing scarring
74
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:
Severe cutaneous blistering hypersensitivity reaction clinically similar to EM but with more frequent mucosal involvement EM Toxic epidermal necrolysis SJS/TEN
75
Stevens-Johnson syndrome/Toxic epidermal necrolysis * Severe cutaneous adverse reaction that affects skin and mucosal surfaces (3)
* 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
Stevens-Johnson syndrome/Toxic epidermal necrolysis * Mainly caused by
drugs but infections and other unidentified risk factors may play a role
77
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
medications < 4 medications or URT infection CD8+ T-cells
78
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
epidermal 10 30 50 Infection
79
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
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
OLP Predisposing Factors (3)
* Lichenoid hypersensitivity reaction * Viral infections * Diabetes Mellitus
81
OLP * Lichenoid hypersensitivity reaction * Clinically & histologically indistinguishable from LP (7)
* Anti-hypertensives * NSAIDs * Statins * Anti-diabetic agents * Amalgam restorations * Cinnamon flavouring agents * Anti-hypothyroidism agents
82
OLP * Viral infections (2)
* HPV * Hep C
83
OLP Clinical Features (3)
* 10-15% have skin LP * 6 P’s * Koebner phenomenon
84
OLP * 6 P’s
* Polygonal * Pruritic * Purple * Papular * Planar * Plaques
85
OLP * Koebner phenomenon (3)
* Wickham striae on flexor surface of wrists * LP lesions at sites of trauma * Does Koebnerization occur in the oral cavity?
86
OLP Clinical Features * Reticular Form (4)
* Classic wickham striae * Bilateral BM * Attached gingiva * Ventral/dorsal tongue
87
OLP * Erosive/Ulcerative form (3)
* Most painful * Yellow fibrin membrane * Higher rate of malignant transformation
88
Desquamative Gingivitis Histopathology (5)
* Hyperkeratosis * Chronic dense band- like infiltrate of lymphocytes * Saw tooth epithelial rete pegs/ridges * Degeneration of basal cell layer * Colloid/Civette bodies
89
Desquamative Gingivitis Hyperkeratosis (2)
* Orthokeratosis * Parakeratosis
90
Desquamative Gingivitis * Colloid/Civette bodies
* degenerating keratinocytes
91
Desquamative Gingivitis Direct Immunofluorescence
* Shaggy/patchy/granu lar deposits of C3 fibrinogen at BMZ
92
Desquamative Gingivitis Management * Cutaneous lesions
* Refer to dermatology
93
skipped Desquamative Gingivitis Management * Topical (7)
* 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
Desquamative Gingivitis Management * Systemic (2)
* Prednisone 1mg/kg (5-7days) * Plaquenil (hydroxychloroquine) 200 mg BID
95
Desquamative Gingivitis Malignant Potential * Mechanisms (3)
* Chronic inflammation * Immune dysregulation * Topical/Systemic immunosuppressants
96
Pemphigus Vulgaris (PV) * * Incidence: --- cases per year * Median Age: * Gender * Mediterranean, South Asian or Jewish heritage * (3) associated with PV
Autoimmune vesicullobullous condition 5/1,000,000 50 yrs No sex predilection HLA-DR4, DRw14 & DQB1
96
Desquamative Gingivitis * Risk (0.1-0.2%) (6)
* 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
* Autoimmune vesicullobullous condition (4)
* Pemphigus subtypes * pemphigus vulgaris * pemphigus vegetans * pemphigus erythematosus * pemphigus foliaceus
98
PV Clinical Features (2)
* Nikolsky sign * application of firm lateral pressure on normal-appearing mucosa adjacent to a pre-existing bulla induces new bulla formation
99
PV Clinical Features * Asboe-Hansen sign
* application of pressure directly to a bulla causes lateral extension
100
PV Oral Features * “first to show, last to go” (3)
* oral lesions 1st sign >50% of cases >1 year * almost all have oral features * most difficult to resolve with tx
101
PV Oral Features * Untreated oral & cutaneous lesions (1)
* persist progressively involving more surfaces
102
PV Histopathology (3)
* Intraepithelial separation * Acantholysis * Lamina propria
103
PV * Acantholysis (2)
* epithelial spinous layer cells fall apart * rounded shape for loose cells (Tzanck cells)
104
PV * Lamina propria (1)
* mild-moderate chronic inflammatory cell infiltrate
105
PV Immunofluorescence Studies * DIF (1) * InDIF & ELISA (2)
* Intercellular IgG/IgM ± C3 * +ve in 80-90% cases * circulating autoantibodies
106
PV Management (5)
* 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
PV * Better prognosis/control if dx early
* before dev of corticosteroid tx 60-90% died due to dehydration, electrolyte imbalances, malnutrition & infection
108
PV * Prednisone (2)
* 5-10% mortality rate now due to complications of long-term corticosteroid use * alternate day prednisone plus steroid-sparing immunosuppressant
109
PV * Steroid-sparing agents (7)
* azathioprine * dapsone * mycophenolate mofetil * cyclophosphamide * IVIg * plasmapheresis * rituximab
110
Bullous Pemphigoid (BP) * - 1 in --- annually * Age: * Gender: * Oral involvement
Most common autoimmune blistering condition 100,000 6th-8th decade 2:1 male uncommon, 8-39%
111
BP Histopathology * Perilesional biopsy (3)
* subepithelial separation * eosinophils within bulla * acute & chronic inflammatory cells
112
BP Direct Immunofluorescence * BMZ (1) * Hemidesmosomes (2)
* linear IgG & C3 (90- 100%) * BP180 (lamina lucida) * BP230
113
BP Indirect Immunofluorescence * Antibody titers (2)
* 50-90% +ve circulating autoantibodies * no correlation with disease activity
114
BP Binding of autoantibodies to BMZ (4)
* starts the complement cascade * degranulation of mast cells * eosinophils & neutrophils recruitment * elastases & matrix metalloproteinases
115
BP Management * Good prognosis (2)
* spontaneous remission after 2-5 years * up to 27% mortality rate
116
BP * Systemic immunosuppressive therapy (2)
* Prednisone QD/QOD * Azathioprine added if no response
117
BP * Alternative therapies (3)
* Dapsone * Tetracycline * Niacinamide
118
BP * Refractory cases (1)
* Prednisone & Cyclophosphamide
119
Mucous Membrane Pemphigoid (MMP) * * Autoantibodies to --- * Positive --- sign * Unknown incidence - 2x as common as -- - most common -- * Age: * Gender:
Chronic, blistering, mucocutaneous autoimmune disease hemidesmosomes Nikolsky PV oral AD 5th-6th decade 2:1 female
120
Sub-types of MMP (4)
* Ocular involvement only * Oral involvement only * Mucosal & cutaneous involvement * Multiple mucosal sites without cutaneous involvement - nose, esophagus, larynx, vagina
121
BP Ocular Complications * Conjunctival mucosal scarring (3)
* Entropion * Symblepahron * Trichiasis
122
BP Ocular Complications * Scarring closes lacrimal gland openings (3) * Blindness (2)
* loss of tears * extremely dry * cornea produces excess keratin * if untreated * opacification from excess keratinization
123
BP Histopathology * Perilesional biopsy (3)
* BM separation * subepithelial clefting * inflammatory infiltrate superficial lamina propria
124
BP Direct Immunofluorescence * Perilesional biopsy (2)
* linear band IgG & C3 (90%) * IgA & IgM (more severe)
125
BP Indirect Immunofluorescence (3)
* MMP lacks detectable circulating autoantibody levels * Anti-epiligrin MMP * Oral MMP only
126
Indirect Immunofluorescence BP * MMP lacks detectable circulating autoantibody levels (1)
* 5-25% pts. +ve
127
Indirect Immunofluorescence BP * Anti-epiligrin MMP (2)
* autoantibodies to epiligrin (laminin-5) * more widespread involvement
128
Indirect Immunofluorescence BP * Oral MMP only (1)
* autoantibodies to a6 integrin
129
BP Management (3)
* Referral to an ophthalmologist * Referral to a dermatologist * Topical corticosteroids (custom trays)
130
* Referral to an ophthalmologist * --% of oral MMP pts develop ocular lesions
25
131
* Referral to a dermatologist * --% of oral MMP pts develop cutaneous lesions
20
132
* Topical corticosteroids (custom trays) (2)
* 0.05% fluocinonide gel BID * 0.05% clobetasol gel BID
133
Paraneoplastic Pemphigus (PNP) (3)
* Neoplasia-induced Pemphigus * Paraneoplastic Autoimmune Multi-organ Syndrome (PAMS) * Rare vesicullobullous disorder - 150 documented cases
134
Paraneoplastic Pemphigus (PNP) * Neoplasm hx (5)
* Non-Hodgkin Lymphoma (42%) * Chronic Lymphocytic Leukemia (29%) * Sarcoma (6%) * Thymoma (6%) * Castleman Disease (6%)
135
PNP Pathogenesis (5)
* 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
* IL-6 produced by lymphocytes in response to patient’s tumor (2)
* IL-6 stimulates abnormal production of antibodies * antibodies against desmosomal complex antigens
137
PNP Clinical Features * PNP developed prior to malignancy dx
* 1/3 of reported cases
138
PNP * Signs & Symptoms (2)
* sudden appearance * polymorphous
139
PNP * Cutaneous lesions (3)
* bullaeerosionstarget lesionslichenoid lesions erythematous papules * papular & pruritic ( cutaneous LP) * palmar or plantar bullae (uncommon in PV)
140
PNP * found where
Vagina, respiratory tract, oral & conjunctival mucosa (scarring similar to MMP)
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PNP Oral Manifestations “Erythema multiformelike stomatitis” (4)
* Hemorrhagic lip crusting * All sites * Oral involvement only in some cases * Multiple painful irregular ulcers
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PNP Histopathology * Non-specific (3)
* lichenoid mucositis * intraepithelial clefting * subepithelial clefting
143
PNP Direct Immunofluorescence (2)
* Weakly +ve linear IgG/C3 along intracellularly & BMZ * InDIF
144
PNP InDIF (5)
* desmoplakin I & II * major bullous pemphigoid antigen * envoplakin * periplakin * desmoglein 1 & 3
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PNP Immunoblotting (2)
* Considered the gold standard for PNP diagnosis * Characteristic reactivity with two plakin proteins is highly sensitive and specific for PNP
146
PNP Management (2)
* High morbidity & mortality (>90%) * Systemic prednisone + immunosuppressant
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PNP * High morbidity & mortality (>90%) * complications from --- lesions * --- therapy * trigger a reactivation of --- * ~ --% develop bronchiolitis obliterans
vesiculobullous immunosuppressive malignant neoplasm 50
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PNP * Systemic prednisone + immunosuppressant (3)
* azathioprine * methotrexate * cyclophosphamide

Dental Management of a Complex Patient (13 decks)

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