Final Flashcards

1
Q

Group of inherited disorders in which TWO or more ectodermally derived structures do not develop normally or fail to develop is:
-Examples of ectoderm structures:

A

Ectodermal Dysplasia

-Skin, hair, nails, teeth, sweat glands

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2
Q

Hypohidrotic Ectodermal Dysplasia causes ______ due to _______

A

Heat intolerance

..reduced sweat glands

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3
Q

What causes fine, sparse blonde hair and oligodontia (less than 6 teeth)

A

Hypohidrotic Ectodermal Dysplasia

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4
Q

When is White Sponge Nevus usually noticed?

  • It is due to:
  • Appearance
A

At birth or early childhood

  • Defect in the normal keratinization of the oral mucosa
  • Asymptomatic, thick, white appearance of the buccal mucosa bilaterally
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5
Q

How do you diagnose White Sponge Nevus?

-Tx:

A
Exfoliative Cytology (better than biopsy)
-No tx necessary
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6
Q

Peutz-Jeghers Syndrome..

  • __% represent new mutations
  • ____ gene is affected
  • Frequency
  • tx
A
  • 35%
  • SKT11
  • 1 in 100,000-200,000
  • genetic counseling
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7
Q

What is characterized by FRECKLE-line lesions on hands, periorifacial skin (mouth, nose, anus, genitals) AND causes GI POLYPS

A

Peutz-Jeghers Syndrome

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8
Q

In Peutz-Jeghers Syndrome (unline Gardner Syndrome), the polyps are NOT ____, but these patients have __x chance of cancer than general population

A

precancerous

-18x

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9
Q

Hereditary Hemorrhagic Telangiectasia

  • What is a telangiectasia?
  • frequency:
  • Due to mutation in a gene that plays a role in:
  • Initial clue to diagnoses
  • Prognosis
A
  • small collection of dilated capillaries
  • 1 in 10,000
  • blood vessel wall integrity
  • frequent epistaxis (nose bleed)
  • Good, but 1-2% mortality dues to blood loss (or brain abscess)
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10
Q

Pt has a 1-2mm red papule on vermillion border of lip that blanch with diascopy, this is:

A

Hereditary Hemorrhagic Telangiectasia

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11
Q

What can cause ArterioVenous problems

A

HHT (Hereditary Hemorrhagic Telangiectasia)

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12
Q

In Pemphigus Vulgaris, ______ destroy ____

A

autoantibodies…desmosomes

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13
Q

Pemphigus

  • age
  • sex
  • prognosis
A
  • 4th-6th decade
  • none
  • fatal if not treated
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14
Q

Pemphigus

  • __% present with oral lesions
  • Appears as:
A
  • 50%

- Ragged erosions and ulcerations

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15
Q

What is the Nikolsky Sign?

-Which disease has it

A
  • Skin rubs off easily

- Pemphigus

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16
Q

In pemphigus, oral lesions are “______ and _____”

A

“First to show and last to go”

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17
Q

In pempigus, direct AND indirect immunoflouresnce (IF) studies will be:

A

positive

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18
Q

Pemphigus tx:

-prognosis

A
SYSTEMIC corticosteriods (NOT topical) often with azathioprene 
-5-10% mortality
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19
Q

Mucous Membrane Pemphigoid is aka:

A

Cicatrical (“scarring”) pemphigoid

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20
Q

Which is 2-4x more common, pemphigus or pemphigoid

A

pemphigoid more common

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21
Q

Pemphigoid..

  • age:
  • sex:
A
  • older (50-60)

- 2:1 female

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22
Q

Pemphigoid may affect:

  • Scarring usually seen with:
  • Often presents as:
  • May see _______ intraorally
A

any mucosal surface

  • conjuctival (symblepharon) lesions
  • desquamative gingivitis
  • intact blisters
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23
Q

With pemphigoid ____ scarring is rare. The most significant aspect of the disease is ____ involvement

A

oral

-ocular

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24
Q

Pemphigoid..

  • What do you submit for biopsy
  • In what solutions?
  • DIF is usually __, IIF is usually __
A
  • Normal mucosa, 0.5-1cm away from ulceration
  • Michel’s and formalin
  • DIF + , IIF -
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25
Pemphigoid tx... - if oral lesions only: - if ocular involvement - fatal?
- oral: topical steroids, tetracycline or dapsone - ocular: immunosuppressive therapy - rarely
26
Bullous pemphigoid.. - Usually affects what age? - ____ lesions are seen primarily, rarely ____ - Initial complaint is usually _____
- Old (75-80) - cutaneous, rarely oral - pruritus (itching)
27
Erythema Multiforme is an ____, self-limiting ulcerative disorder
ACUTE
28
Erythema Multiforme predilection
Young adult female
29
Erythema Multiforme can be caused by
HSV infection
30
Erythema Multiforme minor: | -Major
minor: skin or oral mucosa | - major: at least two mucosal sites plus skin involvement
31
What disease causes Stevens-Johnson and Toxic Epidermal Necrolysis
Erythema Multiforme
32
What causes "target" lesions of skin
Erythema Multiforme
33
Erythema Multiforme... - prognosis for minor: - major: - Toxic Epidermal Necrolysis (TEN):
- minor: good - major: 1-5% mortality - TEN: 25-30% mortality
34
What disease is recurrent in 20% of cases in autumn and spring?
Erythema Mutiforme
35
Erythema Migrans (benign migratory glossitis) is aka: - Occurs in ___ of population (relatively common) - Tx
"geographic tongue" - 1-3% - No tx
36
Pts usually report waxing and waning lesions often seen with fissured tongue (so they might complain from spicy foods)
Erythema Migrans (benign migratory glossitis)
37
Cutaneous Lichen Planus is a chronic _______ disorder
immune-mediated
38
Cutaneous Lichen Planus age range: - Sex - __yr disease course
30-60 - female predilection - 1-2
39
What causes purple pruritic polygonal papules with WICKHAM'S STRIAE
Cutaneous Lichen Planus
40
Cutaneous Lichen Planus usually affects what parts of body?
flexor surfaces of wrists, lumbar region, shins
41
Oral Lichen Planus sex predilection?
-Female
42
Oral Lichen Planus affects which parts of mouth | -Appearance:
Bilateral buccal mucosa, tongue, gingiva | -interlacing white lines
43
Oral Lichen Planus... - most common form: - most symptomatic form
- common: reticular | - symptomatic: erosive
44
What is the most common significant systemic disease? - How many affected in US - Sex? - Average age
Lupus Erythematous - 1.5 million - Female 10x - 31
45
What causes fever, weight loss, arthritis, fatigue, and malaise?
Lupus Erythematous
46
What causes malar "butterfly" rash
Lupus Erythematous
47
What is the most significant complication of Lupus Erythematous? - This organ is involved in __% of patients - What organ is also commonly involved? - __% have vegeatations on these valves
Renal failure - 50% - Heart - 50%
48
__-__% of Lupus Erythematous pts present oral lesions
5-25
49
Lupus Erythematous appears as: | -Area
lichenoid ulcerations/keratotic areas | -palate, buccal mucosa, gingiva
50
Lupus Erythematous tx:
Topical corticosteroids
51
Lupus Erythematous prognosis... - 5 yr survival rate - 20 yr survival rate - Prognosis is worse for:
- 5 = 95% - 20 = 75% - men
52
Chronic Cutaneous Lupus appearance and area: | -Is prognosis better or worse than lupus erythematous
Scaly, erythematous patches on sun-exposed skin of head and neck -much better
53
What is the relatively rare condition characterized by inappropriate deposition of dense collagen
Systemic Sclerosis (scleroderma)
54
Systemic Sclerosis.. - Prevelance (per year) - Sex
- 10-20 per million annually | - Women 3-5x more
55
What causes Raynaud's Phenomenom (deformity of fingers)
Systemic Sclerosis
56
What is the worst complication of Systemic Sclerosis
Causes fibrosis of major organs (pulmonary fibrosis leads to hypertension and heart failure)
57
What causes Microstomia "purse string" appearance of mouth, diffuse widening of PDL, and resorption of posterior mandible and condyle
Systemic Sclerosis
58
Systemic Sclerosis 10yr survival rate with diffuse disease
55-60%
59
CREST is the milder version of _____ | -Sex and age
Systemic Sclerosis | -Women 6th-7th decade
60
What is CREST
Calcinosis cutis, Raynaud's phenomenen, Esophageal dysfunction, Sclerodactyly, Telangiectasia
61
3 highest risk sites for oral cancer (SCC)
- Ventro-lateral tongue - floor of mouth - anterior tonsillar pillars/retromolar area
62
High risk features of oral cancer
larger than 1cm | -non-homogneous patches/plaques
63
What can't Conventional Oral Exam (COE) do?
Can't determine lesions that will progress to cancer
64
Does the ViziLite enhance visual detection of oral cancer beyond COE alone?
NO!
65
VELscope is is good at identifying: - Good at high-risk/low-risk discrimination? - Has a very high false _____ rate
the MARGINS of the lesion - NO! - false positive
66
Which device uses 3 different colors for exam?
Identafi
67
What color does Identafi use to show vasculature
Amber
68
Initial follow-up for suspicious oral lesions should be between __- __
7-21 days