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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Hypohidrotic Ectodermal Dysplasia causes ______ due to _______

A

Heat intolerance

..reduced sweat glands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

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

A

Hypohidrotic Ectodermal Dysplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How do you diagnose White Sponge Nevus?

-Tx:

A
Exfoliative Cytology (better than biopsy)
-No tx necessary
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Peutz-Jeghers Syndrome..

  • __% represent new mutations
  • ____ gene is affected
  • Frequency
  • tx
A
  • 35%
  • SKT11
  • 1 in 100,000-200,000
  • genetic counseling
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

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

A

Hereditary Hemorrhagic Telangiectasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What can cause ArterioVenous problems

A

HHT (Hereditary Hemorrhagic Telangiectasia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

In Pemphigus Vulgaris, ______ destroy ____

A

autoantibodies…desmosomes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Pemphigus

  • age
  • sex
  • prognosis
A
  • 4th-6th decade
  • none
  • fatal if not treated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Pemphigus

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

- Ragged erosions and ulcerations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the Nikolsky Sign?

-Which disease has it

A
  • Skin rubs off easily

- Pemphigus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

In pemphigus, oral lesions are “______ and _____”

A

“First to show and last to go”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A

positive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Pemphigus tx:

-prognosis

A
SYSTEMIC corticosteriods (NOT topical) often with azathioprene 
-5-10% mortality
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Mucous Membrane Pemphigoid is aka:

A

Cicatrical (“scarring”) pemphigoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

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

A

pemphigoid more common

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Pemphigoid..

  • age:
  • sex:
A
  • older (50-60)

- 2:1 female

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

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

A

oral

-ocular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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 -
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Pemphigoid tx…

  • if oral lesions only:
  • if ocular involvement
  • fatal?
A
  • oral: topical steroids, tetracycline or dapsone
  • ocular: immunosuppressive therapy
  • rarely
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Bullous pemphigoid..

  • Usually affects what age?
  • ____ lesions are seen primarily, rarely ____
  • Initial complaint is usually _____
A
  • Old (75-80)
  • cutaneous, rarely oral
  • pruritus (itching)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Erythema Multiforme is an ____, self-limiting ulcerative disorder

A

ACUTE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Erythema Multiforme predilection

A

Young adult female

29
Q

Erythema Multiforme can be caused by

A

HSV infection

30
Q

Erythema Multiforme minor:

-Major

A

minor: skin or oral mucosa

- major: at least two mucosal sites plus skin involvement

31
Q

What disease causes Stevens-Johnson and Toxic Epidermal Necrolysis

A

Erythema Multiforme

32
Q

What causes “target” lesions of skin

A

Erythema Multiforme

33
Q

Erythema Multiforme…

  • prognosis for minor:
  • major:
  • Toxic Epidermal Necrolysis (TEN):
A
  • minor: good
  • major: 1-5% mortality
  • TEN: 25-30% mortality
34
Q

What disease is recurrent in 20% of cases in autumn and spring?

A

Erythema Mutiforme

35
Q

Erythema Migrans (benign migratory glossitis) is aka:

  • Occurs in ___ of population (relatively common)
  • Tx
A

“geographic tongue”

  • 1-3%
  • No tx
36
Q

Pts usually report waxing and waning lesions often seen with fissured tongue (so they might complain from spicy foods)

A

Erythema Migrans (benign migratory glossitis)

37
Q

Cutaneous Lichen Planus is a chronic _______ disorder

A

immune-mediated

38
Q

Cutaneous Lichen Planus age range:

  • Sex
  • __yr disease course
A

30-60

  • female predilection
  • 1-2
39
Q

What causes purple pruritic polygonal papules with WICKHAM’S STRIAE

A

Cutaneous Lichen Planus

40
Q

Cutaneous Lichen Planus usually affects what parts of body?

A

flexor surfaces of wrists, lumbar region, shins

41
Q

Oral Lichen Planus sex predilection?

A

-Female

42
Q

Oral Lichen Planus affects which parts of mouth

-Appearance:

A

Bilateral buccal mucosa, tongue, gingiva

-interlacing white lines

43
Q

Oral Lichen Planus…

  • most common form:
  • most symptomatic form
A
  • common: reticular

- symptomatic: erosive

44
Q

What is the most common significant systemic disease?

  • How many affected in US
  • Sex?
  • Average age
A

Lupus Erythematous

  • 1.5 million
  • Female 10x
  • 31
45
Q

What causes fever, weight loss, arthritis, fatigue, and malaise?

A

Lupus Erythematous

46
Q

What causes malar “butterfly” rash

A

Lupus Erythematous

47
Q

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
A

Renal failure

  • 50%
  • Heart
  • 50%
48
Q

__-__% of Lupus Erythematous pts present oral lesions

A

5-25

49
Q

Lupus Erythematous appears as:

-Area

A

lichenoid ulcerations/keratotic areas

-palate, buccal mucosa, gingiva

50
Q

Lupus Erythematous tx:

A

Topical corticosteroids

51
Q

Lupus Erythematous prognosis…

  • 5 yr survival rate
  • 20 yr survival rate
  • Prognosis is worse for:
A
  • 5 = 95%
  • 20 = 75%
  • men
52
Q

Chronic Cutaneous Lupus appearance and area:

-Is prognosis better or worse than lupus erythematous

A

Scaly, erythematous patches on sun-exposed skin of head and neck
-much better

53
Q

What is the relatively rare condition characterized by inappropriate deposition of dense collagen

A

Systemic Sclerosis (scleroderma)

54
Q

Systemic Sclerosis..

  • Prevelance (per year)
  • Sex
A
  • 10-20 per million annually

- Women 3-5x more

55
Q

What causes Raynaud’s Phenomenom (deformity of fingers)

A

Systemic Sclerosis

56
Q

What is the worst complication of Systemic Sclerosis

A

Causes fibrosis of major organs (pulmonary fibrosis leads to hypertension and heart failure)

57
Q

What causes Microstomia “purse string” appearance of mouth, diffuse widening of PDL, and resorption of posterior mandible and condyle

A

Systemic Sclerosis

58
Q

Systemic Sclerosis 10yr survival rate with diffuse disease

A

55-60%

59
Q

CREST is the milder version of _____

-Sex and age

A

Systemic Sclerosis

-Women 6th-7th decade

60
Q

What is CREST

A

Calcinosis cutis, Raynaud’s phenomenen, Esophageal dysfunction, Sclerodactyly, Telangiectasia

61
Q

3 highest risk sites for oral cancer (SCC)

A
  • Ventro-lateral tongue
  • floor of mouth
  • anterior tonsillar pillars/retromolar area
62
Q

High risk features of oral cancer

A

larger than 1cm

-non-homogneous patches/plaques

63
Q

What can’t Conventional Oral Exam (COE) do?

A

Can’t determine lesions that will progress to cancer

64
Q

Does the ViziLite enhance visual detection of oral cancer beyond COE alone?

A

NO!

65
Q

VELscope is is good at identifying:

  • Good at high-risk/low-risk discrimination?
  • Has a very high false _____ rate
A

the MARGINS of the lesion

  • NO!
  • false positive
66
Q

Which device uses 3 different colors for exam?

A

Identafi

67
Q

What color does Identafi use to show vasculature

A

Amber

68
Q

Initial follow-up for suspicious oral lesions should be between __- __

A

7-21 days