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Oral Path Final > Immune-Mediated Mucocutaneous Disorders > Flashcards

Immune-Mediated Mucocutaneous Disorders Flashcards

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1
Q

What is the autoimmune condition that has inappropriate production of Abs by host against tissue that destroys desmosomes?

A

Pemphigus Vulgaris

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2
Q

What do desmosomes due, and what happens to them as a result of Pemphigus Vulgaris

A

Desmosomes bond epithelial cells together , Abs inhibit adherence so a split develops in the epithelium

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3
Q

How common, what is sex predilection and average age of people affected with Pemphigus Vulgaris

A

Rare, no sex predilection, average age of 50

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4
Q

What are severe results of untreated Pemphigus Vulgaris that can result in fatality

A
  • Severe infection
  • Loss of fluids/electrolyes
  • Malnutrition due to mouth pain
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5
Q

How often are oral lesions seen with Pemphigus Vulgaris and how do they present

A
  • > 50%, superficial, raged erosions and ulcerations
  • Any oral mucosal surface
  • Intact oral blisters are rarely seen
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6
Q

What are skin features of Pemphigus Vulgaris, and what is a key diagnostic sign

A
  • Flaccid bullae on skin

- Positive Nikolsky sig, (Inducing a bulal by applying firm, lateral pressure to normal appearing skin)

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7
Q

When do oral manifestations appear/resolve with Pemphigus Vulgaris

A
  • Initial manifestation and most difficult to resolve
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8
Q

What is immunofluorescence?

A

Technique that uses fluorescent labeled Abs to detect specific targets

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9
Q

What are Direct/Indirect immunofluoresecence, and are they positive or negative for Pemphigus Vulgaris

A
  • Direct- Used to detect aut-Abs bounds to patient’s tissue
  • Indirect- Used to detect auto-Abs circulating in the blood
  • Both positive
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10
Q

What tissue should be sampled in Pemphigus Vulgaris for DIF

A
  • Tissue adjacent to ulceration
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11
Q

Auto-abs in Pemphigus Vulgaris bind to what components

A
  • Desmoglein 1 and 3
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12
Q

What disease shows intraepithelila clefting above basal layer and acanytholosis?

A

Pemphigus Vulgaris

- Acanthylosis is breakdown of spinous layer

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13
Q

What is the treatment and prognosis for Pemphigus Vulgaris

A
  • Systemic corticosteroids w/ steroid sparing agent

- Before steroid therapy (60-90% mortality), now 5-10% usually as complication to therapy

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14
Q

What disease usually presents as desquamative gingivitis (and what is desquamative gingivitis)

A

Mucous Membrane Pemphigoid

- Descriptive term for erythema and ulcerative appearance

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15
Q

What is another name for Mucous Membrane Pemphigoid

A

Cicatricial pemphigoid

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16
Q

How common is Mucous Membrane Pemphigoid and what does other disease does it resemble

A

Pemphigus vulgaris (Mucous Membrane Pemphigoid is 2x more common the pemphigus)

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17
Q

What age and sex are most commonly affected by Mucous Membrane Pemphigoid

A

2:1 F, 50-60

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18
Q

What surfaces can be affected in Mucous Membrane Pemphigoid, where is scarring usually see/not seen

A
  • Any mucosal surface, sometimes skin

- Scarring usually seen w/ conjunctival and cutaneous lesions

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19
Q

Can Mucous Membrane Pemphigoid present w/ oral tissues as the only affeceted site? And are intact blisters usually seen?

A
  • Yes, intraoral blisters are common, but scarring is rare
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20
Q

What is the most significant aspect of Mucous Membrane Pemphigoid and what does it cause?

A
  • Ocular involement- Scarring obsturcts orifices of glands that produce tear film- Dry eye
  • Dry eye leads to keritinization of the corneal epithelium, leads to blindness
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21
Q

Where is the cleft fomration for Mucous Membrane Pemphigoid

A
  • Subepithelial cleft formation (epithelium separated from CT at the BMZ)
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22
Q

Why must a sample from the periphery of the lesion be taken with Mucous Membrane Pemphigoid and how far away should the sample be from the ulcerated area

A
  • Epithelium easily strips off, .5-1 cm away
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23
Q

What should tissue of Mucous Membrane Pemphigoid be submitted in?

A
  • Michel’s solution and formalin
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24
Q

Where are immunoreactants with Mucous Membrane Pemphigoid and are DIF and IIF positive

A
  • Linear deposition at BMZ

- Positive DIF, negative IFF (5-25% have circulating auto-Abs)

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25
What is the treatment for Mucous Membrane Pemphigoid?
- Oral lesions only- Topical steroid, tetracycline, or dapsone, and frequent prophylaxis - Systemic immunosuppressive therapy w/ ocular involvement
26
Prognosis for Mucous Membrane Pemphigoid
- Rarely fatal, can be controlled | - Blindness, rare undergoes spontaenous resolution
27
What is primarily seen with Bullous Pemphigoid and how often are oral tissues involved
Cutaneous lesion primarily, oral lesions (8-39%)
28
What is the initial complaint with Bullous Pemphigoid and what age group is affected?
- Pruritus followed by cutaneous blisters | - Older
29
Where is the cleft and are DIF and IIF positive w/ Bullous Pemphigoid
- Subepithelial | - Both positive (immunoreactants along BMZ)
30
Treatment of Bullous Pemphigoid
Similar treatment as Mucous Membrane Pemphigoid | Can spontaneously resolve in 1-2 years
31
What disease is self limiting (2-6 weeks) ulcerative disorder w/ and acute onset
Erythema Multiforme
32
What population does Erythema Multiforme affect
- Young adult, 20s-30s
33
What are the 3 etiologies of Erythema Multiformeand their frequency
- 50% unkown - 25% preceding viral or bacterial infection - 25% medication related (antibiotic or analgesic)
34
What are the 3 spectrums of Erythema Multiforme
1) EM minor- Skin or mucosa only 2) EM Major- Stevens-Johnson syndrome, 2 mucosal sites plus skin involvement 3) Toxis Epidermal Necrolysis- Lyell's disease, diffuse bullous involvement of skin and mucosa
35
What are the oral features associated with Erythema Multiforme
- Hemorrhagic crusting of lips - Widespread oral ulcers w/ ragged margins - LM, BM, and tongue affected, gingiva and hard palate usually spared - Target skin lesions
36
Can light microscopy or immunofluorescence diagnose Erythema Multiforme
- No, characteristic only
37
What disease usually shows keratinocyte destruction, subepithelial edema, miced inf. infiltrate, preivascular inf?
Erythema Multiforme
38
Treatment for all 3 forms of Erythema Multiforme
- Supportive care for mild cases (steroid syrup, analgeiscs) - EM Minor- Corticosteroids, but most likely would heal anyways - Stevens-Johnson- D/C toxic drug - Toxic Epidermal Necrolysis- Burn unit, IV pooled Ig, avoid corticosteroid
39
How often is Erythema Multiforme recurrent and what can the cause be?
- 20% | - HSV or drug, antivirals can help prevent recurrences
40
Prognosis for Erythema Multiforme
- Good for mild moderate - EM major- 2-10% mortality - TEN- 34% mortality, improving with Ig therapy
41
What disease show well-demarcated zones of eythema surrounded at least partially by a slightly elevated yellow-white serpentine or scalloped border of the tongue
Erythema Migrans
42
What are 2 other names for Erythema Migrans
- Benign migratory glossitis | - Geographic tongue
43
How common is Erythema Migrans and what surface does it usually affected, and how does it present
- Common (1-3% of population) - Dorsal and lateral ant. 2/3rds of tongue - Waxes and wanes
44
What other condition is often seen with Erythema Migrans?
Geographic tongue
45
What is Erythema Migrans called of it develops on other non-keratinzed mucosal surfaces?
Ectopic geographic tongue
46
What disease's biopsy shows parakeratosis w/ extensive microabscess formation in superficial spinous later, shearing of parakeratin, and reamining epithelium being thinner, diagnosed as psoriasiform mucositis
Erythema Migrans
47
Treatment of Erythema Migrans
- None, can be sensitive to hot foods | - May respond to stronger topical corticosteroid
48
Age, sex, and symtpoms, and location for Cutenous Lichen Planus
- Adults 30-60 - Female - Purple polygonal prurutic papules w/ Wickham's striae - Flexor of wrists, lumbar region, and shins
49
What are the 2 forms of Oral Lichen Planus?
1) Reticular- Interlacing white lines, most common, dorsal tongue may appear as patchy keratosis/atrophy 2) Erosive- Shallow ulcers, peripheral eythema and radiating white lines, more symptomatic
50
What age/sex and locations does Oral Lichen Planus affect
- Adults, 3:2 females - Bilateral BM, tongue, gingiva - May occur with skin lesions
51
What are the microscopic findings associated with Oral Lichen Planus, and can it establish the diagnosis
- Hyperkeratosis, alternating atrophy and thickening of spinous layer, absent rete ridges, degeneration of basal cell layer, band-like infiltrates of lymphocytes - Clinical not microscopic diagnosis
52
Treatment for Oral Lichen Planus
- Treatment of possible superimposed candidiasis - Reticular- None - Erosive- Topical corticosteroid
53
Prognosis for Oral Lichen Planus
- Good, no molecular evidence of malignant transformation
54
Term for a number of conditions that can mimic Oral Lichen Planus clinically and hisopathologically?
Lichenoid Mucositis
55
Most common significant immune-mediated systemic disease
Systemic Lupus Erythematosus
56
How many people are affected in U.S., and what sex/age is more common
- 1.5 million | - Women 8-10:1, colored, 31
57
What are the signs and symptoms of Systemic Lupus Erythematosus
- Protean early (fever, weight loss, fatigue) - Malar butterfly rash sparing nasolabial folds - Skin lesions that flare with sun exposure
58
How often are cardiac and renal involvement seen with Systemic Lupus Erythematosus, and what are the complications
- Renal- 40-50%, main concern w/ disease is kidney failure | - Cardiac- Common, pericarditis, 50% have sterile vegetations on heart valves (Libman-Sacks endocarditis)
59
How often are oral lesions w/ Systemic Lupus Erythematosus and how do the present
- 5-25% - Non specific or lichenoid, affect palate, BM, gingiva - Respond to corticosteroid
60
What pattern does Systemic Lupus Erythematosus show microscopically
Perivascular inf
61
What does the Lupus band test show regarding Systemic Lupus Erythematosus
- Positive test- Deposition of band of immunoreactans at basement membrane zone of normal skin, not specific for Systemic Lupus Erythematosus, and sometimes negative
62
How often do serum studies show anti-nuclear antibodies w/ Systemic Lupus Erythematosus, is it a specific finding, and how many of these are directed toward double stranded DNA
- 95% of patients - Non-specific, could be useful for screening - 70% at double stranded DNA (these are more specific)
63
Treatment for Systemic Lupus Erythematosus
- Decrease UV light exposure | - Non-steroidal anti-inf, anti-malarial, systemic corticosteroids for more significant cases
64
Prognosis for Systemic Lupus Erythematosus
- 95% 5 year - 75% 15 year - Most common COD is renal failure, worse prognosis for men
65
What disease resembles Systemic Lupus Erythematosus and almost exclusively affects skin and mucosa?
Chronic Cutaneous Lupus
66
What do oral lesions of Chronic Cutaneous Lupus resemble
- Erosive LP, wax and wane
67
What appears on sun-exposed skin for patients with Chronic Cutaneous Lupus
- Scaly, erythematous patches, that scar or atrophy and cause a cosmentic problem
68
How do oral lesions present microscopically with Chronic Cutaneous Lupus
- Lichenoid mucositic and vasculitis
69
Are anti-nuclear antibodies present in serologic studies with Chronic Cutaneous Lupus
No
70
Treatment for Chronic Cutaneous Lupus
- Avoid UV light | - Topical corticosteroids, anti-malarial drugs or low-dose thalidomide for more difficult cases
71
Prognosis for Chronic Cutaneous Lupus
- 5% may progress to SLE - Lesions confined to skin - 50% resolve after several years
72
What is a condition characterized by inappropriate deposition of dense collagen?
Systemic Sclerosis
73
How common and what age/sex affected by Systemic Sclerosis
- Rare (20 million annually), 3:1 females , adults
74
What is Raynaud's Phenomenon and is it specific for Systemic Sclerosis
- Discoloration of fingers/toes after exposure to temp change or emotional events, not specific
75
What other conditions involving the hands are seen with Systemic Sclerosis
- Claw like deformity of fingers, ulceration of finger tips | - Acro-osteolysis (destruction of digit tips, including bone)
76
How does the skin feel with Systemic Sclerosis
- Diffuse, smooth, hard texture
77
What other systems are affected by Systemic Sclerosis and which is a common cause of death
- Pulmonary, renal, GI fiborisis, pulmonary hypertension, and heart failure (COD)
78
What are the oral manifestations of Systemic Sclerosis
- Microstomia, pinched "purse-string) appearance - Dysphagia w/ esophagael involvement - Resoprtion of post. ramus, condyle, coronoid process or chin in 10-20% - Sometimes tooth resportion
79
What disease show dense collagen on biopsy of affected organ, auto-Abs against SCl-70 (topoisomerase I)
Systemic Sclerosis
80
Treatment for Systemic Sclerosis
- No definitive - Supportive care of hypertension and dysphagia - 30-50% survival after 8 years
81
What is a milder variant of Systemic Sclerosis and what does it stand for
CREST Sydrome (Calcinosis Cutis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia)
82
What sex/age is commonly affected by CREST Syndrome
- Women, 6th-7th decade
83
What are Auto-Abs directed against in CREST Syndrome
Anticentromere Abs
84
Treatment/Prognosis for CREST Syndrome
- Systemic care similar to Systemic Sclerosis | - 80% 6 year, 50% 12 year

Oral Path Final (4 decks)

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