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

Developmental Conditions (4) Flashcards

(94 cards)

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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
What is the treatment for hereditary hemorrhagic telangiectasia (HHT)?
Genetic counseling Mild - no treatment Moderate - selective cryotherapy or cautery of bothersome lesions Severe - septal dermoplasty to prevent epistaxis
26
T/F: Pemphigus vulgaris has an autoimmune etiology.
True
27
In patients with PV, autoantibodies destroy ________.
desmosomes inhibit epithelial adherence resulting in split in epithelium
28
T/F: Oral lesions are rare in patients with PV.
False > 50% "first to show, last to go"
29
Where should a biopsy be taken for a suspected PV lesion?
At the periphery of the lesion
30
What is a histopathological feature of PV?
Intraepithelial clefting above basal layer
31
What are the two types of immunofluorescence?
Direct - detect ABs bound to patient tissue Indirect - detect ABs in the blood Both will be + in patient with PV
32
What is the treatment/prognosis for PV?
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
33
T/F: Mucous membrane pemphigoid is twice as common as pemphigous vulgaris.
True
34
T/F: PV is more common in females.
False MMP
35
T/F: MMP causes a separation of tissue within the epithelium.
False Subepithelial split
36
T/F: Topical steroids are an effective treatment for PV.
False Systemic steroids
37
Blindness due to keratinization of corneal epithelium from dry eyes is a significant aspect of __________.
mucous membrane pemphigoid (MMP)
38
T/F: PV causes separation of epithelium from the basement membrane.
False MMP
39
What must be included in a biopsy for MMP or PV?
Generous sample of normal tissue
40
What is the treatment and prognosis for MMP?
Oral lesions: topical steroids and frequent prophylaxis Refer to ophthalmologist for follow up Usually controlled and rarely fatal
41
T/F: PV is the most common of the autoimmune blistering conditions.
False Bullous pemphigoid (BP)
42
What is the average age of a patient with BP?
75-80 y.o.
43
T/F: Oral involvement with BP is common.
False
44
Bullous pemphigoid shows subepithelial clefting similar to which other condition?
MMP
45
Which immunopathological features are seen with BP?
Positive DIF and IIF
46
T/F: Most BP cases resolve spontaneously in 1-2 years.
True
47
What is the etiology for Erythema multiforme?
50% - unknown 25% - preceding infection (herpes or mycoplasma pneumoniae) 25% - med-related (antibiotics and analgesics)
48
What age group is typically affected with Erythema Multiforme?
20s-30s
49
T/F: Patients with EM will show prodromal symptoms ~1 week before onset.
True Included fever, malaise, headache, etc.
50
What are the clinical features of EM minor?
Ulcers on extremities and mucosa Hemorrhagic crusting of vermilion zones
51
What is the most common skin lesion seen with EM minor?
“Target lesions” on extremities Although there can be many appearances
52
T/F: The gingiva and hard palate are the most commonly affected mucosal areas in EM minor.
False Gingiva and palate often spared Other surfaces will have erythematous patches which undergo necrosis -> ulcers with irregular borders
53
What are the characteristics of EM major?
2 or more mucosal sites in conjunction with skin lesions
54
What is the difference between Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)?
SJS - <10% skin involvement and usually younger TEN - >30% skin involvement and usually over 60 y.o.
55
What are some distinguishing features between EM and SJS/TEN?
SJS/TEN almost always triggered by drug SJS/TEN skin lesions on trunk not extremities
56
T/F: DIF and IFF are useful in diagnosing between EM, SJS, and TEN.
False Can rule out other immune-mediated conditions
57
What is the treatment for EM and SJS/TEN?
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
Of EM, TEN, and SJS, which disease has the highest mortality rate?
TEN 25-30%
59
What is another term for erythema migrans?
Geographic tongue
60
What causes the erythema in geographic tongue?
Atrophy of filiform papillae and shearing off of parakeratin
61
What are some clinical features of erythema migrans?
Occurs in 1/3 of patients with fissured tongue Wax and wanes Heals then develops in different area
62
What is the most common area for erythema migrans?
Dorsal and lateral anterior 2/3 of tongue *Can be seen in other areas of the mouth, not just tongue
63
What is the appearance of erythema migrans?
Well-demarcated zones of erythema surrounded by slightly elevated yellow-white border
64
What is the prognosis for erythema migrans?
Good Benign process
65
T/F: Cutaneous lichen planus has a female predilection.
True
66
T/F: Cutaneous lichen planus may resolve within 7-10 years.
True
67
What are the clinical features of cutaneous lichen planus?
Purple polygonal pruritic papules with Wickham’s striae Seen on wrists, lumbar region, shins
68
What are the two forms of oral lichen planus?
1. Reticular - lacy white lines 2. Erosive - erythematous, may ulcerate * Reticular most common
69
Hyperkeratosis and pointed, “saw toothed” rete ridges are histo features of which immune-mediated disease?
Oral lichen planus
70
T/F: Oral lichen planus can be diagnosed based on histo features.
False Clinical diagnosis
71
T/F: Systemic steroids are used to treat oral ELP.
False Topical steroids and treat candidiasis if present
72
Several conditions can mimic oral lichen planus such as drug reaction, cinnamon reaction, amalgam reaction, etc. These conditions are termed _____________.
lichenoid mucositis
73
What is the most common collagen vascular/connective tissue disease in the U.S.?
Lupus erythematosus
74
What are the three forms of lupus?
1. Chronic cutaneous lupus erythematosus (CCLE) 2. Systemic lupus Erythematosus (SLE) 3. Subacute cutaneous lupus erythmatosis (SCLE)
75
Which form of lupus is also known as “discoid lupus”?
CCLE
76
What are the features of the mucosa in a patient with CCLE?
Lichenoid mucositis, but almost always with skin lesions in sun exposed areas Painful esp with acidic, salty, spicy foods
77
T/F: Females are affected 8-10 times more than males with CCLE.
False SLE
78
What is the most at risk population for systemic lupus erythematosus?
Black women around 30 y.o.
79
What is the initial manifestation of systemic lupus?
Fever, weight loss, fatigue
80
What is the butterfly rash?
Rash that is on the face but spares the nasolabial folds Seen in systemic lupus
81
What is the most significant aspect of systemic lupus?
Renal involvement (40-50% of patients) Cardiac involvement also common
82
How many patients with systemic lupus have oral involvement?
5-25% Lupus cheilitis in vermilion zones
83
Serum studies show _________ present in 95% of SLE cases, negative in CCLE.
Anti-nuclear antibodies (ANAs)
84
What is the prognosis for CCLE?
Good
85
What is the prognosis for SLE?
SLE Worse for men than women Worse for blacks than whites
86
___________ is a rare immune-mediated condition where dense collagen replaces and destroys normal tissue.
Systemic sclerosis
87
Sclerodactyly, Raynaud’s phenomenon and acro-osteolysis are clinical features seen in the hands of patients with which immune-mediated disease?
Systemic sclerosis
88
T/F: Raynaud’s phenomenon is specific for systemic sclerosis.
False
89
What are some radiographic features of systemic sclerosis?
Widening of PDL Resorption of posterior ramus, condyle, etc. Root resorption
90
Which antibodies are often seen with systemic sclerosis?
Autoantibodies against Scl-70 Anticentromere antibodies
91
T/F: Steroids are often prescribed for systemic sclerosis.
False
92
What is the most common cause of death in systemic sclerosis?
Pulmonary involvement
93
What is the milder variant of systemic sclerosis often seen in women in their 6th-7th decade?
CREST syndrome ``` Calcinosis cutis Raynaud’s phenomenon Esophageal dysfunction Sclerodactyly Telangiectasia ```
94
T/F: CREST syndrome has a better prognosis than systemic sclerosis.
True

AU18: Oral Path 2 (18 decks)

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