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AU18: Oral Path 2 > Developmental Conditions (4) > Flashcards

Developmental Conditions (4) Flashcards

1
Q

What are the four major developmental mucocutaneous conditions?

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

What is the major diagnosing factor for ectodermal dysplasia?

A

Two or more ectodermally derived structures do not develop normally or fail to develop

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3
Q

What are the major body parts that are affected by ectodermal dysplasia?

A

Skin, hair, nails, teeth, sweat glands

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4
Q

_____________ is one of the best known types of ectodermal dysplasia. Patients often have heat intolerance and the features are more pronounced in males than females.

A

Hypohidrotic ectodermal dysplasia

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5
Q

What are the intraoral clinical features of hypohidrotic ectodermal dysplasia?

A

Hypodontia or oligodontia; conical crowns; xerostomia of varying degrees

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6
Q

What is the treatment for hypohidrotic ectodermal dysplasia?

A

Genetic counseling; fixed, removable, implants, ortho, etc.

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7
Q

Which disorder can mimic ectodermal dysplasia clinically?

A

Polygenetic oligodontia

missing teeth but the remaining teeth are normal and hair/nails are typically normal

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8
Q

____________ is a genetically determined skin disorder due to a defect in the normal keratinization of the oral mucosa.

A

White sponge nevus

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9
Q

T/F: White sponge nevus will appear later in life.

A

False

Birth or early childhood

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10
Q

What are the intraoral affects of white sponge nevus?

A

Thick, white appearance of buccal mucosa bilaterally

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11
Q

T/F: Exfoliative cytology is often used to diagnose white sponge nevus.

A

True

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12
Q

What will a biopsy show with white sponge nevus?

A

Perakeratosis with acanthosis (thickened spinous layer)

Perinuclear eosinophilic condensation of cytoplasm

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13
Q

What is the treatment for white sponge nevus?

A

Treatment not needed

Tetracycline rinses seem to help

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14
Q

Which gene is mutated with Peutz-Jeghers syndrome?

A

STK11

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15
Q

T/F: Peutz-Jeghers syndrome involves benign polyps of the gastrointestinal tract.

A

True

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16
Q

T/F: Peutz-Jeghers syndrome puts patients at 18 times higher risk of developing cancer.

A

True

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17
Q

What are some intraoral symptoms of Peutz-Jeghers syndrome?

A

Hyperpigmented macules of lips and oral mucosa

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18
Q

T/F: The gastrointestinal polyps in Peutz-Jeghers syndrome are precancerous lesions.

A

False

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19
Q

What is the treatment for Peutz-Jeghers syndrome?

A

Genetic counseling; monitor for intussusception and tumor development

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20
Q

What is telangiectasia?

A

Small collection of dilated capillaries

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21
Q

What are the clinical features of hereditary hemorrhagic telangiectasia (HHT)?

A

Frequent spontaneous epistaxis

Numerous 1mm-2mm red papules

May be seen in mucosa and skin

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22
Q

Patients with _______ are at greater risk for arteriovenous fistulas affecting the lungs, liver, or brain.

A

HHT

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23
Q

What locations orally have pronounced lesions from HHT?

A

Vermillion zones, tongue, buccal mucosa

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24
Q

Diagnosis of HHT requires three of the following four features:

A
  1. Recurrent epistaxis
  2. Telangiectasias of mucosa and skin
  3. AV malformation involving lung, liver, or brain
  4. Family history of HHT
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25
Q

What is the treatment for hereditary hemorrhagic telangiectasia (HHT)?

A

Genetic counseling

Mild - no treatment

Moderate - selective cryotherapy or cautery of bothersome lesions

Severe - septal dermoplasty to prevent epistaxis

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26
Q

T/F: Pemphigus vulgaris has an autoimmune etiology.

A

True

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27
Q

In patients with PV, autoantibodies destroy ________.

A

desmosomes

inhibit epithelial adherence resulting in split in epithelium

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28
Q

T/F: Oral lesions are rare in patients with PV.

A

False

> 50%
“first to show, last to go”

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29
Q

Where should a biopsy be taken for a suspected PV lesion?

A

At the periphery of the lesion

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30
Q

What is a histopathological feature of PV?

A

Intraepithelial clefting above basal layer

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31
Q

What are the two types of immunofluorescence?

A

Direct - detect ABs bound to patient tissue

Indirect - detect ABs in the blood

Both will be + in patient with PV

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32
Q

What is the treatment/prognosis for PV?

A

Systemic corticosteroids w/ azathioprine

30% resolve on their own after 10 years

Can be fatal if not treated

Mortality due to long term steroid use

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33
Q

T/F: Mucous membrane pemphigoid is twice as common as pemphigous vulgaris.

A

True

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34
Q

T/F: PV is more common in females.

A

False

MMP

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35
Q

T/F: MMP causes a separation of tissue within the epithelium.

A

False

Subepithelial split

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36
Q

T/F: Topical steroids are an effective treatment for PV.

A

False

Systemic steroids

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37
Q

Blindness due to keratinization of corneal epithelium from dry eyes is a significant aspect of __________.

A

mucous membrane pemphigoid (MMP)

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38
Q

T/F: PV causes separation of epithelium from the basement membrane.

39
Q

What must be included in a biopsy for MMP or PV?

A

Generous sample of normal tissue

40
Q

What is the treatment and prognosis for MMP?

A

Oral lesions: topical steroids and frequent prophylaxis

Refer to ophthalmologist for follow up

Usually controlled and rarely fatal

41
Q

T/F: PV is the most common of the autoimmune blistering conditions.

A

False

Bullous pemphigoid (BP)

42
Q

What is the average age of a patient with BP?

A

75-80 y.o.

43
Q

T/F: Oral involvement with BP is common.

44
Q

Bullous pemphigoid shows subepithelial clefting similar to which other condition?

45
Q

Which immunopathological features are seen with BP?

A

Positive DIF and IIF

46
Q

T/F: Most BP cases resolve spontaneously in 1-2 years.

47
Q

What is the etiology for Erythema multiforme?

A

50% - unknown
25% - preceding infection (herpes or mycoplasma pneumoniae)
25% - med-related (antibiotics and analgesics)

48
Q

What age group is typically affected with Erythema Multiforme?

49
Q

T/F: Patients with EM will show prodromal symptoms ~1 week before onset.

A

True

Included fever, malaise, headache, etc.

50
Q

What are the clinical features of EM minor?

A

Ulcers on extremities and mucosa

Hemorrhagic crusting of vermilion zones

51
Q

What is the most common skin lesion seen with EM minor?

A

“Target lesions” on extremities

Although there can be many appearances

52
Q

T/F: The gingiva and hard palate are the most commonly affected mucosal areas in EM minor.

A

False

Gingiva and palate often spared

Other surfaces will have erythematous patches which undergo necrosis -> ulcers with irregular borders

53
Q

What are the characteristics of EM major?

A

2 or more mucosal sites in conjunction with skin lesions

54
Q

What is the difference between Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)?

A

SJS - <10% skin involvement and usually younger

TEN - >30% skin involvement and usually over 60 y.o.

55
Q

What are some distinguishing features between EM and SJS/TEN?

A

SJS/TEN almost always triggered by drug

SJS/TEN skin lesions on trunk not extremities

56
Q

T/F: DIF and IFF are useful in diagnosing between EM, SJS, and TEN.

A

False

Can rule out other immune-mediated conditions

57
Q

What is the treatment for EM and SJS/TEN?

A

EM - discontinue causative drug, systemic/topical steroids early, IV re-hydration, topical anesthetic or analgesic

SJS/TEN - All same except no steroids
*NSAIDS thought to cause TEN

58
Q

Of EM, TEN, and SJS, which disease has the highest mortality rate?

A

TEN 25-30%

59
Q

What is another term for erythema migrans?

A

Geographic tongue

60
Q

What causes the erythema in geographic tongue?

A

Atrophy of filiform papillae and shearing off of parakeratin

61
Q

What are some clinical features of erythema migrans?

A

Occurs in 1/3 of patients with fissured tongue

Wax and wanes

Heals then develops in different area

62
Q

What is the most common area for erythema migrans?

A

Dorsal and lateral anterior 2/3 of tongue

*Can be seen in other areas of the mouth, not just tongue

63
Q

What is the appearance of erythema migrans?

A

Well-demarcated zones of erythema surrounded by slightly elevated yellow-white border

64
Q

What is the prognosis for erythema migrans?

A

Good

Benign process

65
Q

T/F: Cutaneous lichen planus has a female predilection.

66
Q

T/F: Cutaneous lichen planus may resolve within 7-10 years.

67
Q

What are the clinical features of cutaneous lichen planus?

A

Purple polygonal pruritic papules with Wickham’s striae

Seen on wrists, lumbar region, shins

68
Q

What are the two forms of oral lichen planus?

A
  1. Reticular - lacy white lines
  2. Erosive - erythematous, may ulcerate
    * Reticular most common
69
Q

Hyperkeratosis and pointed, “saw toothed” rete ridges are histo features of which immune-mediated disease?

A

Oral lichen planus

70
Q

T/F: Oral lichen planus can be diagnosed based on histo features.

A

False

Clinical diagnosis

71
Q

T/F: Systemic steroids are used to treat oral ELP.

A

False

Topical steroids and treat candidiasis if present

72
Q

Several conditions can mimic oral lichen planus such as drug reaction, cinnamon reaction, amalgam reaction, etc. These conditions are termed _____________.

A

lichenoid mucositis

73
Q

What is the most common collagen vascular/connective tissue disease in the U.S.?

A

Lupus erythematosus

74
Q

What are the three forms of lupus?

A
  1. Chronic cutaneous lupus erythematosus (CCLE)
  2. Systemic lupus Erythematosus (SLE)
  3. Subacute cutaneous lupus erythmatosis (SCLE)
75
Q

Which form of lupus is also known as “discoid lupus”?

76
Q

What are the features of the mucosa in a patient with CCLE?

A

Lichenoid mucositis, but almost always with skin lesions in sun exposed areas

Painful esp with acidic, salty, spicy foods

77
Q

T/F: Females are affected 8-10 times more than males with CCLE.

78
Q

What is the most at risk population for systemic lupus erythematosus?

A

Black women around 30 y.o.

79
Q

What is the initial manifestation of systemic lupus?

A

Fever, weight loss, fatigue

80
Q

What is the butterfly rash?

A

Rash that is on the face but spares the nasolabial folds

Seen in systemic lupus

81
Q

What is the most significant aspect of systemic lupus?

A

Renal involvement (40-50% of patients)

Cardiac involvement also common

82
Q

How many patients with systemic lupus have oral involvement?

A

5-25%

Lupus cheilitis in vermilion zones

83
Q

Serum studies show _________ present in 95% of SLE cases, negative in CCLE.

A

Anti-nuclear antibodies (ANAs)

84
Q

What is the prognosis for CCLE?

85
Q

What is the prognosis for SLE?

A

SLE

Worse for men than women

Worse for blacks than whites

86
Q

___________ is a rare immune-mediated condition where dense collagen replaces and destroys normal tissue.

A

Systemic sclerosis

87
Q

Sclerodactyly, Raynaud’s phenomenon and acro-osteolysis are clinical features seen in the hands of patients with which immune-mediated disease?

A

Systemic sclerosis

88
Q

T/F: Raynaud’s phenomenon is specific for systemic sclerosis.

89
Q

What are some radiographic features of systemic sclerosis?

A

Widening of PDL

Resorption of posterior ramus, condyle, etc.

Root resorption

90
Q

Which antibodies are often seen with systemic sclerosis?

A

Autoantibodies against Scl-70

Anticentromere antibodies

91
Q

T/F: Steroids are often prescribed for systemic sclerosis.

92
Q

What is the most common cause of death in systemic sclerosis?

A

Pulmonary involvement

93
Q

What is the milder variant of systemic sclerosis often seen in women in their 6th-7th decade?

A

CREST syndrome

Calcinosis cutis
Raynaud’s phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasia
94
Q

T/F: CREST syndrome has a better prognosis than systemic sclerosis.

AU18: Oral Path 2 (18 decks)

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