Chapter 16 Dermatologic Diseases Flashcards

1
Q

What is a group of inherited conditions in which two or more ectodermal derived anatomic structures fail to develop?

A

-Ectodermal dysplasia

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2
Q

What are some common things that fail to develop in ectodermal dysplasia?

A
  • Skin
  • Hair
  • Nails
  • Teeth
  • Sweta glands
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3
Q

What is the best known type of Ectodermal Dysplasia?

A

-Hypohidrotic ectodermal dysplasia

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4
Q

T/F Hypohidrotic ectodermal dysplasia is an X-linked female predominant disease

A

False

-Male predominant

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5
Q

What type of hair does a person with hyphohidrotic ectodermal dysplasia?

A
  • Fine

- Sparse hair

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6
Q

What are the shape of teeth in someone with ectodermal dysplasia?

A

-Conical shaped

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7
Q

What is white sponge nevus?

A

-Genodermatosis a genetically determined skin disorder

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8
Q

White sponge nevus has a defect in what keratins?

A
  • 4

- 13

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9
Q

T/F White sponge nevus is autosomal recessive

A

False

-Autosomal dominant

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10
Q

What age do white sponge nevus lesions appear first?

A

-Birth or early childhood

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11
Q

What does a white sponge nevus look like?

A

-Symmetrical thickened white corrugated diffuse plaque on the bilateral buccal mucosa

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12
Q

Where do you see a Warty Dyskeratoma?

A

-Skin or oral mucosa

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13
Q

What is Peutz-Jeghers Syndrome characterized by?

A
  • Freckle-like lesions of the hands, perioral skin and oral mucosa
  • Intestinal polyposis
  • Predisposition for affected patients to develop cancer
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14
Q

If the intestinal polyps are premalignant what syndrome is that associated with?

A

-Gardner’s syndrome

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15
Q

Patients with Peutz-Jeghers syndrome have a problem with intestinal obstruction due to what?

A

-Intussusception

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16
Q

T/F Pts are about 18x more likely to have a malignancy in their lifetime if diagnosed with Peutz-Jeghers syndrome

A

True

17
Q

What are the oral lesions of Peutz-Jeghers syndrome?

A

-1 to 4 mm brown macules

18
Q

where do the the oral lesions of Peutz-Jeghers occur?

A
  • Labial mucosa
  • Buccal mucosa
  • Tongue
19
Q

What are the CNS manifestations of Tuberous Sclerosis?

A
  • Mental retardation
  • Seizure disorders
  • Potato-like growths called tubers
20
Q

Besides the CNS manifestations what else do you see in Tuberous Sclerosis?

A
  • Angiofribromas of the skin
  • Ungual fibromas
  • Shagreen patches
  • Ash-leaf spot
21
Q

What are facial angiofibromas?

A

-Smooth surfaced papules occuring primarily on the nasolabial fold area

22
Q

What are ASh-leaf spots?

A

-Ovoid areas of hypopigmentation

23
Q

What is a rare tumor of the heart muscle that is also associated with Tuberous Sclerosis?

A

-Rhabdomyoma

24
Q

What are the oral manifestations with Tuberous Sclerosis?

A
  • Developmental enamel pitting on the facial aspect of anterior permanent teeth
  • Multiple fibrous papules
25
Q

What are the four diseases of Pemphigus?

A
  • Pemphigus vulgaris
  • Pemphigus vegetans
  • Pemphigus erythematosus
  • Pemphigus foliaceus
26
Q

What is the first sign of pemphigus vulgaris?

A

-Oral lesions

27
Q

What are the autoantibodies directed against in pemphigus vulgaris (autoimmune)?

A
  • Desmosomes (Desmoglome 3)

- Causes an intraepithelial split

28
Q

What is a characteristic feature of pemphigus vulgaris?

A

-Positive Nikolsky sign

29
Q

What is a nikolsky sign?

A

-You can induce a Bulla if firm lateral pressure is exerted

30
Q

What are the loose cells called in Pemphigus vulgaris?

A

-Tzanck cells (these are also found in herpes)

31
Q

The cells of the epithelium fall apart in pemphigus vulgaris what is that termed?

A

-Acantholysis

32
Q

If you do a direct immunofluorescence in pemphigus vulgaris what is it positive for?

A
  • C3

- IgG (both found within the epithelium

33
Q

What do you use to treat Pemphigus vulgaris?

A

-Systemic corticosteroid

34
Q

What is Epidermolysis bullosa acquisita associated with?

A

Type VII collagen of anchoring fibrils with a sub basement membrane split