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AU 16- Oral Path Exam 4 > Developmental Mucocutaneous Disease > Flashcards

Developmental Mucocutaneous Disease Flashcards

1
Q

Ectodermal dysplasia, White sponge Nevus, Peutz-Jeghers Syndrome, and Hereditary hemorrhagic telangiectasia are all _____ mucocutaneous diseases.

A

Developmental

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2
Q

______ is a group or inherited disorders in which ____ or more ectodermally derived structures do not develop normally or fail to develop.

A

Ectodermal Dysplasia

TWO

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3
Q

Name examples of ectodermally derived structures.

A 
skin
hair
nails
teeth
sweat glands
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4
Q

True or False: There are several patterns of inheritance for ectodermal dysplasia.

A

True, depends on the type (over 170 subtypes)

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5
Q

What is a common form of ectodermal dysplasia?

What other condition could be mistakenly diagnosed as Ectodermal Dysplasia?

A

Hypohidrotic Ectodermal Dysplasia

–polygenetic oligodontia–

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6
Q

True or False: Hypohidrotic ED patients frequently have cold intolerance.

A

False, heat intolerance due to reduced sweat glands

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7
Q

Describe a typical Hypohidrotic ED patient’s hair.

A

fine
sparse
blonde or light colored
thin eyebrows/eyelashes

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8
Q

What is the typical oral presenation of ED patients?

A

lack of development of 6 or more teeth (oligodontia)

conical teeth

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9
Q

How is ED “managed”?

A

Genetic counseling

prosthetic management: dentures, overdentures, fixed appliances, dental implants`

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10
Q

White Sponge Nevus is an autosomal ________ condition.

A

dominant (relatively rare)- genodermatosis

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11
Q

What causes white sponge nevus? When is it first noticed?

A

a defect in the normal keratinization of the oral mucosa

first noticed at birth or early childhood, sometimes adolescence

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12
Q

What is the clinical presentation of White Sponge Nevus?

A

asymptomatic, thick, white appearance of the buccal mucosa bilaterally
other oral sites, nasal, esophageal, laryngeal, or anogenital mucosa may be involved also

looks very similar to morsicatio buccarum

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13
Q

What are the histopathologic features of White Sponge Nevus?

A
  • parakeratosis with acanthosis (thickening of the spinous layer)
  • epithelial cells often show perinuclear eosinophilic condensation of cytoplasm (pathognomonic)
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14
Q

_______ cytology is more diagnostic than a biopsy sample when determining white sponge nevus.

A

exfoliative

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15
Q

What is the pathognomonic histo feature of white sponge nevus?

A

perinuclear eosinophilic condensation of cytoplasm

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16
Q

How is White Sponge Nevus treated?

A

treatment is not necessary

  • reassure the patient that it is harmless
  • good prognosis
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17
Q

Peutz-Jeghers Syndrome is an autosomal ______ condition that affects the ______ gene.

A

dominant (rare condition)
SKT11

PJ is a skater (SKT) boy (11)

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18
Q

What does the SKT11 gene encode for?

A

serine/threonine kinase
S = serine
K = kinase
T = threonine

19
Q

What percentage of PJ Syndrome is due to a new mutation?

A

35%

20
Q

What is the clinical presentation of Peutz-Jeghers Syndrome?

A
  • usually recognized in childhood as freckle-like lesions which develop on the hands, periorificial skin, and oral mucosa
  • also, polyps develop in the GI tract (jejunum and ileum in particular)
21
Q

What is “periorificial” skin?

A

mouth
nose
anus
genital

22
Q

True or False: Peutz-Jeghers syndrome can cause bowel obstruction.

A

True

23
Q

How does Peutz-Jeghers cause bowel obstruction? Is surgery needed?

A

intussusception (“telescoping” of a proximal segment into a distal segment)

-may self correct but surgery is warranted to prevent ischemic necrosis

24
Q

True or False: Like Gardner Syndrome, polyps associated with Peutz-Jeghers are precancerous.

A

False,
PJ polyps = not precancerous
(benign hamartomatous growths of intestinal glandular epithelium)

25
Q

Do Peutz-Jeghers Syndrome patients have an increased susceptibility to cancer?

A

yes, about 18 times greater than a control population

GI, pancreas, breast, ovary, genital tract

26
Q

What is the treatment for Peutz-Jeghers Syndrome?

A
  • genetic counseling

- monitoring for intussusception or tumor development

27
Q

Hereditary Hemorrhagic Telangiectasia is an autosomal ______ disorder. What is the frequency?

A

dominant

1 in 10,000

28
Q

HHT is due to a mutation of one of ____ different genes, each of which play a role in ___ _____ ____ integrity and share similar clinical features.

A

two

blood vessel wall

29
Q

What is telangiectasia?

A

small collection of dilated capillaries

30
Q

What is the initial “clue” in diagnosing HHT?

A

frequent spontaneous epistaxis (nose bleeds)

31
Q

Where are HHT lesions most commonly found?

A

on vermilion zone of lips, tongue, and buccal mucosa

32
Q

What is the clinical appearance of HHT lesions?

A

numerous 1-2mm red papules on oropharyngeal/nasal mucosa that blanch with diascopy

33
Q

True or False: HHT telangiectasias may be seen on the hands, feet and conjunctiva.

A

True

34
Q

What percentage of patients with HHT have arteriovenous fistulas that affect the lungs? liver? brain?

A

30% lungs
30% liver
10-20% brain

35
Q

How many features are evaluated when diagnosing HHT? How many of those must the patient have?

A

4 features

-must have 3 of 4 for HHT diagnosis

36
Q

What are the four HHT features?

A
  • recurrent/spontaneous epistaxis
  • telangiectasias of mucosa and skin
  • AV malformation involving the lung, liver, or brain
  • family history of HHT
37
Q

Histopathologically, HHT patients have a collection of ___ ____ blood vessels in the _____ connective tissue.

A

thin-walled

superficial

38
Q

What is the treatment for HHT?

A
  • genetic counseling
  • mild HHT = no treatment
  • moderate HHT = selective cryotherapy or electrocautery of bothersome lesions
  • severe HHT = septal dermoplasty to prevent epistaxis
39
Q

If an HHT patient has significant GI involvement and blood loss, what is the necessary treatment?

A
  • iron replacement

- transfusion (if necessary)

40
Q

True or False: Antibiotic prophylaxis prior to dental treatment is suggested for all HHT patients.

A

False

41
Q

True or False: Antibiotic prophylaxis prior to dental treatment is suggested for HHT patients with an AV fistula involving the brain.

A

True
dental procedures that cause bacteremia
1% prevalence of brain abscess in these HHT patients

42
Q

What is the prognosis of HHT?

A

generally good

1-2% morality is sometimes noted due to complications with blood loss

43
Q

If a brain abscess develops, what is the anticipated mortality rate?

A

10%

despite early diagnosis and appropriate treatment

AU 16- Oral Path Exam 4 (6 decks)

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