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AU'18 Oral Path II > Developmental and Immune-mediated Mucocutaneous Conditions - part I > Flashcards

Developmental and Immune-mediated Mucocutaneous Conditions - part I Flashcards

1
Q

categories of mucocutaneous conditions

A
  1. developmental

2. immune-mediated

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2
Q

developmental mucocutaneous conditions

A
  1. ectodermal dysplasia
  2. white sponge nevus
  3. Peutz-Jeghers syndrome
  4. Hereditary hemorrhagic telangiectasia
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3
Q

immune-mediated mucocutaneous conditions

A
  1. pemphigus vulgaris
  2. mucous membrane pemphigoid
  3. bullous pemphigoid
  4. erythema multiforme
  5. erythema migrans (geographic tongue)
  6. linchen planus
  7. lichenoid mucositis
  8. lupus erthematosus
  9. systemic sclerosis
  10. CREST syndrome
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4
Q

ectodermal dysplasia is a group of inherited disorders in which what?

A

two or more ectodermally derived structures do not develop normally or fail to develop (hypoplasia or aplasia)

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5
Q

T/F: there are several patterns of inheritance depending on the type of ectodermal dysplasia

A

true, ~200 different subtypes

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6
Q

pts with ectodermal dysplasia have either hypoplasia or aplasia of what?

A
  1. skin
  2. hair
  3. nails
  4. teeth
  5. sweat glands
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7
Q

what is one of the best known types of ectodermal dysplasia?

A

hypohidrotic ectodermal dysplasia

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8
Q

clinical features of hypohidrotic ectodermal dysplasia

A
  1. heat intolerance
  2. fine, sparse blond or light color hair, eyebrows, eyelashes
  3. hypodontia
  4. oligodontia
  5. conical roots
  6. periocular hyperpigmentation
  7. protuberant lips (midface hypoplasia)
  8. prominent forehead
  9. varying degrees of xerostomia
  10. dystrophic or brittle nails
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9
Q

why do pts with hypohidrotic ectodermal dysplasia experience heat intolerance??

A

because they lack sweat glands

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10
Q

T/F: features of pts with hypohidrotic ectodermal dysplasia are more pronounced in females than males

A

false, more pronounced in males than females

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11
Q

oligodontia

A

lack of development of 6 or more teeth

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12
Q

what type of roots do pts with hypohidrotic ectodermal dysplasia have?

A

conical

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13
Q

radiographic features of hypohidrotic ectodermal dysplasia

A
  1. hypodontia/oligodontia
  2. conical roots
  3. abnormally-shaped crowns (conical, tapered, pointed, smaller)
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14
Q

tx of hypohidrotic ectodermal dysplasia

A
  1. genetic counseling pts and parents

2. tx plan may include fixed, removable, implants, ortho, etc. depends on individual case

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15
Q

T/F: polygenetic oligodontia is another name for ectodermal dysplasia

A

FALSE

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16
Q

dental findings of pts with polygenetic oligodontia can mimic what?

A

ectodermal dysplasia

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17
Q

pts w/ polygenetic oligodontia vs. ectodermal dysplasia

A
  1. missing teeth but morphology of remaining teeth in pts with polygenetic oligodontia is normal
  2. normal hair, eyebrows, eyelashes
  3. normal fingernails
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18
Q

what can help reduce the error of misdiagnosing pts with ectodermal dysplasia instead of polygenetic oligodontia?

A

genetic testing

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19
Q

what causes white sponge nevus?

A

defect in the normal keratinization of the oral mucosa

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20
Q

white sponge nevus is classified as what?

A

genodermatosis

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21
Q

genodermatosis

A

genetically determined skin disorder

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22
Q

T/F: white sponge nevus is common

A

false, rare

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23
Q

white sponge nevus is autosomal what?

A

dominant with variable expressivity

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24
Q

when does white sponge nevus appear?

A

at birth or early childhood, sometimes adolescence

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25
Q

clinical features of white sponge nevus

A
  1. asymptomatic

2. thick, white appearance of buccal mucosa bilaterally

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26
Q

what other sites may be affected by white sponge nevus?

A
  1. nasal
  2. esophageal
  3. laryngeal
  4. anogenital mucosa
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27
Q

keratin from white sponge nevus is stained what color?

A

yellow

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28
Q

histo of white sponge nevus

A

parakeratosis with acanthosis

2. perinuclear eosinophilic condensation of cytoplasm is pathognomonic

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29
Q

parakeratosis with acanthosis

A

thickening of spinous layer

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30
Q

what is sometimes more diagnostic of white sponge nevus than scalpel biospy?

A

exfoliative cytology

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31
Q

tx of white sponge nevus

A

none unless cosmetic concern

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32
Q

what is reported to help in tx’ing white sponge nevus?

A

tetracycline rinses

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33
Q

T/F: pts should be reassured that white sponge nevus is a harmless condition

A

true

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34
Q

px of white sponge nevus

A

good

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35
Q

T/F: Peutz-Jeghers syndrome is rare but well-recognized

A

true

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36
Q

when is Peutz-Jeghers syndrome usually noted?

A

in childhood

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37
Q

Peutz-Jeghers syndrome is autosomal what?

A

dominant (~35% new mutations)

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38
Q

mutation of what gene allows uncontrolled cell growth?

A

STK11 gene

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39
Q

clinical features of Peutz-Jeghers syndrome

A
  1. BENIGN hamartomatous polyps of gastrointestinal tract, esp jejunum and ileum
  2. hyperpigmented macules of lips and oral mucosa
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40
Q

pts with Peutz-Jeghers syndrome experience bowel obstruction due to what?

A

intussusception

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41
Q

intussusception

A

“telescoping” of proximal segment into distal segment

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42
Q

when does the bowel problems of pts with Peutz-Jeghers syndrome become evident?

A

3rd decade (i.e. 20’s)

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43
Q

pts with Peutz-Jeghers syndrome are at a high risk of developing cancer how many times greater than the control population?

A

18x

44
Q

type of cancers more likely to develop in males with Peutz-Jeghers syndrome

A
  1. GI tract

2. pancreas

45
Q

type of cancers more likely to develop in females with Peutz-Jeghers syndrome

A
  1. genital tract
  2. ovary
  3. breast
46
Q

T/F: hyperpigmented macules found on pts with Peutz-Jeghers syndrome may fade with age

A

true

47
Q

what other sites may hyperpigmented macules be found on pts with Peutz-Jeghers syndrome?

A
  1. eyes
  2. nostrils
  3. anus
  4. hands
  5. feet
48
Q

T/F: the gastrointestinal polyps found in pts with Peutz-Jeghers syndrome are precancerous

A

false, NOT precancerous

49
Q

the gastrointestinal polyps found in pts with Peutz-Jeghers syndrome are considered what?

A

benign growths of intestinal glandular epithelium

50
Q

tx of Peutz-Jeghers syndrome

A
  1. genetic counseling for parents and pt

2. monitor for intussusception and for tumor development

51
Q

intussusception in pts with Peutz-Jeghers syndrome may self-correct or may require surgery to prevent what?

A

ischemic necrosis

52
Q

what happens if cancer develops in pts with Peutz-Jeghers syndrome?

A

tx appropriately

53
Q

telangiectasia

A

small collection of dilated capillaries

54
Q

is hereditary hemorrhagic telangiectasia (HHT) uncommon?

A

yes

55
Q

hereditary hemorrhagic telangiectasia (HHT) is autosomal what?

A

dominant

56
Q

what causes hereditary hemorrhagic telangiectasia (HHT)?

A

mutation of one of two genes which are responsible for blood vessel wall integrity

57
Q

what be an initial clue to dx’ing hereditary hemorrhagic telangiectasia (HHT)?

A

frequent spontaneous epistaxis

58
Q

clinical features of hemorrhagic telangiectasia (HHT)

A
  1. numerous 1-2 mm red papules blanch with diascopy

2. telangiectasias on mucosa and skin, including hands and feet

59
Q

other sites that hemorrhagic telangiectasia (HHT) may appear on?

A
  1. oral
  2. oropharyngeal
  3. nasal
  4. genitourinary
  5. conjunctival mucosa
  6. GI mucosa
60
Q

arteriovenous fistulas in pts with hemorrhagic telangiectasia (HHT) may affect what?

A
  1. lungs (30% of pts)
  2. liver (30%)
  3. brain (10-20%)
61
Q

what is often the most dramatic and most easily identified on pts with hemorrhagic telangiectasia (HHT)?

A

oral lesions

62
Q

location of oral lesions due to hemorrhagic telangiectasia (HHT)

A
  1. vermilion zones
  2. tongue
  3. buccal mucosa
63
Q

dx of hemorrhagic telangiectasia (HHT) requires 3 of what 4 features?

A
  1. reccurent spontaneous epistaxis
  2. telangiectasias of mucosa and skin
  3. AV malformation involving the lung, liver or brain
  4. family hx of HHT
64
Q

histo features of hemorrhagic telangiectasia (HHT)

A

collection of thin-walled blood vessels in the superficial CT

65
Q

tx of MILD hemorrhagic telangiectasia (HHT)

A
  1. genetic counseling for parents and child

2. no tx

66
Q

tx of MODERATE hemorrhagic telangiectasia (HHT)

A
  1. genetic counseling for parents and child

2. selective cryotherapy or electrocautery bothersome lesions

67
Q

tx of severe hemorrhagic telangiectasia (HHT)

A
  1. genetic counseling for parents and child

2. septal dermoplasty to prevent epistaxis

68
Q

px of hemorrhagic telangiectasia (HHT)

A

good

69
Q

1-2% mortality is noted in pts with hemorrhagic telangiectasia (HHT) because of what?

A

complications related to blood loss

70
Q

if a brain abscess develops in a pt with hemorrhagic telangiectasia (HHT), what percent mortality can be anticipated?

A

10% despite early dx and appropriate tx

71
Q

etiology of pemphigus vulgaris (PV)

A

autoimmune

72
Q

Pemphigus vulgaris (PV) is due to inappropriate production of what?

A

antibodies by the host directed against host tissue (autoantibodies)…

damage to host by host’s own immune response

73
Q

Pemphigus vulgaris (PV) autoantibodies destroy what?

A

desmosomes

74
Q

role of desmosomes

A

bond epithelial cells together

75
Q

what happens when antibodies inhibit desmosomes?

A

inhibits adherence so a split develops in the epithelium

76
Q

T/F: Pemphigus vulgaris (PV) is relatively rare

A

true, ~5 cases per million dx’d each year in general population

77
Q

what is the average age of pts dx’d with pemphigus vulgaris (PV)?

A

50 y.o.

78
Q

what is the gender predilection for pemphigus vulgaris (PV)?

A

no gender predilection

79
Q

oral lesions in pts with pemphigus vulgaris (PV)

A
  1. superficial, ragged erosions and ulcerations

2. any mucosal surface

80
Q

T/F: the oral lesions in pts with pemphigus vulgaris (PV) are the “first to show, last to go”

A

true, oral lesions are the initial manifestation of the disease and the most difficult to resolve with treatment

81
Q

clinical features of pemphigus vulgaris (PV)

A
  1. flaccid vesicles, bullae on skin

2. + Nikolsky sign

82
Q

+ Nikolsky sign

A

inducing a bulla by applying firm, lateral pressure to normal appearing skin

83
Q

what is rarely seen intact intraorally in pts with pemphigus vulgaris (PV)?

A

flaccid vesicles, bullae

84
Q

what is important in managing pemphigus vulgaris (PV)?

A

plaque control

85
Q

normal tissue adjacent to ulceration or erosion of pemphigus vulgaris (PV) should be sampled for what?

A
  1. direct immunoflorescent (in Michel’s solution)

2. light microscopic evaluation

86
Q

histo of pemphigus vulgaris (PV)

A
  1. INTRAEPITHELIAL clefting above the basal layer (i.e. within the epithelium)
  2. acantholysis
87
Q

acantholysis

A

breakdown of spinous layer; cells appear to fall apart

88
Q

what remains attached to the CT seen microscopically in pemphigus vulgaris (PV)?

A

only basal cell layer of epithelium

89
Q

immunopathology identifies what?

A

immune responses associated with diseases

90
Q

immunofluorescence

A

technique that uses fluorescent-labeled antibodies to detect specific targets

91
Q

T/F: immunopathology is used to help identify several immune-mediated conditions

A

true

92
Q

direct immunofluorescence (DIF) is used to detect what?

A

autoantibodies bound to pt’s tissues

93
Q

indirect immunofluorescence (IIF) is used to detect what?

A

antibodies circulating in the blood

94
Q

immuno features of pemphigus vulgaris (PV)

A
  1. both DIF and IIF studies will be positive in pemphigus vulgaris
  2. autoantibodies bind desmosomal components (desmoglein 1 & 3)
95
Q

DIF-immunoreactants are what in pemphigus vulgaris (PV)?

A

in intercellular areas b/w the surface epithelial cells “chicken wire” with SUPRAbasilar cleft

96
Q

tx of pemphigus vulgaris (PV)

A

systemic corticosteroids - often azathiprine or other steroid-sparing agents

97
Q

why does topical corticosteroids have little effect in tx’ing pemphigus vulgaris (PV)?

A

PV is a systemic disease

98
Q

what percent of pemphigus vulgaris (PV) cases will resolve on their own after 10 years?

A

~30%

99
Q

T/F: pemphigus vulgaris (PV) is usually fatal if not tx’d

A

true

100
Q

why may pemphigus vulgaris (PV) be fatal if not tx’d?

A
  1. severe infection
  2. loss of fluids/electrolytes
  3. malnutrition due to mouth pain
101
Q

prior to corticosteroid therapy, what mortality rates of pemphigus vulgaris (PV) cases were what percent?

A

60-90%

102
Q

what may lead to mortality of pts with pemphigus vulgaris (PV)?

A

complications of long-term steroid

103
Q

what is the morality rate of pts with pemphigus vulgaris (PV) today?

A

5-10% mortality

104
Q

mortality today in pts with pemphigus vulgaris (PV) is usually due to what?

A

complications of therapy (side effect of steroids, immune-suppression)

105
Q

what drug is used to tx pemphigus vulgaris (PV) suppresses bone marrow and is a carcinogen?

A

azathioprine

AU'18 Oral Path II (31 decks)

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