Ch 16 Oral Dermatology Flashcards

1
Q

what parts of the body may be affected by ectodermal dysplasia?

A

-skin, hair, nails, teeth and sweat glands

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2
Q

skin problems of ectodermal dysplasia?

A
  • fine, sparse blond hair
  • reduced eyebrow and eyelash hair
  • periocular hyperpigmentation
  • dystrophic brittle nails
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3
Q

what are the oral problems/manifestations of ectodermal dysplasia?

A
  • xerostomia secondary to decrease salivary gland development
  • hypodontia
  • cone shaped teeth
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4
Q

which disorder has cone shaped teeth, hypodontia, and xerostomia?

A

-oral manifestations of ectodermal dysplasia

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5
Q

what is the treatment for ectodermal dysplasia?

A
  • genetic couseling for the family

- prosthetic replacement of the teeth

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6
Q
  • thick white buccal mucosa bilaterally

- may also be seen in other mucosa

A

white sponge nevus (AD disorder)

-benign hyperparakeratotic condition with no txt

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7
Q

benign hyperparakeratotic condition with no treatment

A

white sponge nevus

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8
Q

-AD disorder seen in NC Lumbi indians

A

Hereditary Benign Intraepithelial Dyskeratosis

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9
Q
  • thick white plaques on the buccal mucosa and conjuctiva

- dyskeratosis in upper layers of epithelium

A

Hereditary Benign Intraepithelial Dyskeratosis

-benign, no txt

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10
Q
  • Erythematous pruritic papules on the trunk and scalp that have foul odor
  • AD
  • nails show ridges and splits
  • oral: white papules
  • keratolytic agents
A

Darier’s Disease (Keratosis Follicularis)

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11
Q

-orally manifestation: single white papule located on hard palate or alveolar ridge

A
  • Warty Dyskeratoma (Isolated Darier’s Disease)
  • txt: excision
  • identical histology to darier’s disease with suprabasilar cleft and dyskeratosis
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12
Q
  • Freckly-like lesion in and around the oral cavity and hands
  • intestinal polyposis with predisposition to change into adenocarcinoma
A

Peutz-Jeghers Syndrome

-genetic counseling

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13
Q

location of intestinal polyps in peutz-jeghers syndrome?

A

polyps in jejunum and ileum

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14
Q
  • vascular hamartomas due to decreaded blood vessel wall integrity
  • frequent epistaxis
  • telangiectasia intraoral, hands, feet, GI tract, GU tract and eye
  • iron deficiency anemia if severe
A

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu Syndrome)

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15
Q
  • multiple variations of abnormal collagen production due to genetic abnormalities
  • patients have hypermobility of joints and elasticity of the skin (“rubberman”)
A

Ehlers-Danlos Syndrome

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16
Q
  • 50% can touch their nose with their tongue
  • Subluxation of the TMJ
  • Bruising and bleeding of the oral tissues
A

Ehlers-Danlos Syndrome

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17
Q

20% of these lesions are caused by drug reactions, the others we don’t know the cause

A

Lichen Planus

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18
Q

chronic inflammatory disease

-white striations, papules or plaques on the buccal mucosa, tongue and gingiva

A

lichen planus

19
Q

age rage and sex predilection for lichen planus?

A

45-60 Women 2:1, skin lesions common

20
Q

what is thought to be a possibility for lichen planus?

A

thought to be T-cell mediated autoimmune disease in which CD8+T cells trigger apoptosis of oral epithelial cells

21
Q

patients with lichen planus with persistent mouth sores are at increased risk to get…

A

SCCa

22
Q

-Allergic or immunologic reaction
-20% medication induced
-Linked to chronic hepatic diseases especially Hepatitis C
-Psycogenic (stress and anxiety)
-Associated with LP of skin, HBP
and diabetes
-Dental materials

A

causes of lichen planus

23
Q
  • purple
  • polygonal
  • pruitic
  • papules (papular)
A

skin lichen planus

24
Q
  • used to diagnose pemphigus
  • dislodgement of skin by lateral pressure (or air syring)
  • used for multiple diseases
A

Nikolsky Sign

25
Q

BMMP

A

Benign Mucous Membrane Pemphigoid

26
Q
  • average age 60
  • women 2:1
  • oral lesions (conjunctival, nasal, esophageal, laryngeal and vaginal
  • clinical vesticles and bullae
  • Ocular involvement most significant (may leave to blindness, and 25% one eye before the other)
A

BMMO (Benign Mucous Membrane Pemphigoid)

27
Q

what are the eye findings of BMMP?

A

25% oral develop ocular:

  • symblepharons (adhesions between upper and lower eyelid)
  • entropion (scarring/eyelids turn inward)
  • trichiasis (eyelashes rub cornea and globe and scarring closes openings of lacrimal glands)
28
Q

Histology: subepithelial slefting

-Imuno Fluorescence to basement membrane area

A

BMMP

29
Q

treatment for BMMP

A
  • Dapsone (cannot be G6PD deficient)
  • Steroids
  • Tetracycline and niacinamide
  • immunosuppresive agents
30
Q

two types of pemphigus that affect oral tissues

A
  • pemphigus vulgaris

- pemphigus vegetans

31
Q
  • a mucocutaneous AI disease characterized by intraepithelial acanthoslysis due to immune complex deposition at cellular attachment bridges
  • intraepithelial clefting (seaparation)
  • blistering due to AI attack of desmosomes
A

pemphigus vulgaris

  • txt: steroids
  • 60-80% will die without txt
32
Q

average age for pemphigus vulgaris

  • sex predilection
  • race predilection
  • location
A
50
-male=female
-jewish
-ragged erosions and ulcers: ANY oral mucosa
(positive Nkolsky sign)
33
Q

“the oral lesions are the first to show and the last to go”

A

pemphigus vulgaris

34
Q

Histology: intraepithelial separation just above the basal cell layer leaving “row of tombstones”

  • Acontholsysis with floating Tzanck cells
  • positive indirect immunofluorescence for intercellular IgG, IgM and C3
A

Pemphigus Vulgaris

35
Q
  • Cross-reacting antibodies in lymphoma or leukemia attack desmosomal complex.
  • May precede the discovery of the underlying malignancy.
  • Often fatal
A

Paraneoplastic Pemphigus (Neoplasia-Induced Pemphigus)

36
Q
  • occurs in recipients of allogenic bone marrow transplantation
  • engrafted cells recognize body as foreign
A

Graft Versus Host Disease

37
Q
  • few weeks after transplant
  • affects 50%
  • mild rash to TEN
  • diarrhea, nausea, vomiting, abdominal pain and liver dysfunction
A

acute graft versus host disease

38
Q

-lasts 100 days to years and
develops 33-64%
-Mimics autoimmune disease
-Skin lesions resemble LP or systemic sclerosis

A

chronic GVHD

39
Q

oral manifestations of GVHD

A
  • 33-75% of AGVHD and 80% of CGVHD
  • Resembles lichen planus
  • Some complain of burning sensation (R/O candidiasis
  • xerostomia common
  • immunologic response destroying salivary glands
  • mucoceles of soft palate
40
Q

txt of GVHD

A

-Prophylactic therapy with immunomodulatory and immunsuppressive agents such as cyclosporine & prednisone
-Methotrexate has been added and reduced GVHD further
-Thalidomide has shown some
promise
-Topical CS oral lesions
-Psoralen and Ultraviolet A (PUVA) improve lichenoid form
-Treat xerostomia

41
Q

-Increased proliferative activity of cutaneous keratinocytes
-Immunologic with activated
T-lymphocytes
-Well demarcated silvery plaque with silvery scale on surface
-Non-symptomatic to itching

A

Psoriasis

-no txt

42
Q

histology:

  • parakeratosis with elongated rete ridges
  • Munroe abscesses
  • perivacular chronic inflammation
  • CT papillae dilated capullaries close to epithelial surface
A

Psoriasis

43
Q

lupus erythematoosus

A

-