Ch 16 Oral Dermatology

Question 1

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

Answer 1

-skin, hair, nails, teeth and sweat glands

Question 2

skin problems of ectodermal dysplasia?

Answer 2

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

Question 3

what are the oral problems/manifestations of ectodermal dysplasia?

Answer 3

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

Question 4

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

Answer 4

-oral manifestations of ectodermal dysplasia

Question 5

what is the treatment for ectodermal dysplasia?

Answer 5

• genetic couseling for the family

- prosthetic replacement of the teeth

Question 6

• thick white buccal mucosa bilaterally

- may also be seen in other mucosa

Answer 6

white sponge nevus (AD disorder)

-benign hyperparakeratotic condition with no txt

Question 7

benign hyperparakeratotic condition with no treatment

Answer 7

white sponge nevus

Question 8

-AD disorder seen in NC Lumbi indians

Answer 8

Hereditary Benign Intraepithelial Dyskeratosis

Question 9

• thick white plaques on the buccal mucosa and conjuctiva

- dyskeratosis in upper layers of epithelium

Answer 9

Hereditary Benign Intraepithelial Dyskeratosis

-benign, no txt

Question 10

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

Answer 10

Darier’s Disease (Keratosis Follicularis)

Question 11

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

Answer 11

• Warty Dyskeratoma (Isolated Darier’s Disease)
• txt: excision
• identical histology to darier’s disease with suprabasilar cleft and dyskeratosis

Question 12

• Freckly-like lesion in and around the oral cavity and hands
• intestinal polyposis with predisposition to change into adenocarcinoma

Answer 12

Peutz-Jeghers Syndrome

-genetic counseling

Question 13

location of intestinal polyps in peutz-jeghers syndrome?

Answer 13

polyps in jejunum and ileum

Question 14

• 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

Answer 14

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu Syndrome)

Question 15

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

Answer 15

Ehlers-Danlos Syndrome

Question 16

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

Answer 16

Ehlers-Danlos Syndrome

Question 17

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

Answer 17

Lichen Planus

Question 18

chronic inflammatory disease

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

Answer 18

lichen planus

Question 19

age rage and sex predilection for lichen planus?

Answer 19

45-60 Women 2:1, skin lesions common

Question 20

what is thought to be a possibility for lichen planus?

Answer 20

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

Question 21

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

Answer 21

SCCa

Question 22

-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

Answer 22

causes of lichen planus

Question 23

• purple
• polygonal
• pruitic
• papules (papular)

Answer 23

skin lichen planus

Question 24

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

Answer 24

Nikolsky Sign

Question 25

BMMP

Answer 25

Benign Mucous Membrane Pemphigoid

Question 26

- 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)

Answer 26

BMMO (Benign Mucous Membrane Pemphigoid)

Question 27

what are the eye findings of BMMP?

Answer 27

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)

Question 28

Histology: subepithelial slefting | -Imuno Fluorescence to basement membrane area

Answer 28

BMMP

Question 29

treatment for BMMP

Answer 29

- Dapsone (cannot be G6PD deficient) - Steroids - Tetracycline and niacinamide - immunosuppresive agents

Question 30

two types of pemphigus that affect oral tissues

Answer 30

- pemphigus vulgaris | - pemphigus vegetans

Question 31

- 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

Answer 31

pemphigus vulgaris - txt: steroids - 60-80% will die without txt

Question 32

average age for pemphigus vulgaris - sex predilection - race predilection - location

Answer 32

``` 50 -male=female -jewish -ragged erosions and ulcers: ANY oral mucosa (positive Nkolsky sign) ```

Question 33

"the oral lesions are the first to show and the last to go"

Answer 33

pemphigus vulgaris

Question 34

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

Answer 34

Pemphigus Vulgaris

Question 35

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

Answer 35

Paraneoplastic Pemphigus (Neoplasia-Induced Pemphigus)

Question 36

- occurs in recipients of allogenic bone marrow transplantation - engrafted cells recognize body as foreign

Answer 36

Graft Versus Host Disease

Question 37

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

Answer 37

acute graft versus host disease

Question 38

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

Answer 38

chronic GVHD

Question 39

oral manifestations of GVHD

Answer 39

- 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

Question 40

txt of GVHD

Answer 40

-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

Question 41

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

Answer 41

Psoriasis | -no txt

Question 42

histology: - parakeratosis with elongated rete ridges - Munroe abscesses - perivacular chronic inflammation - CT papillae dilated capullaries close to epithelial surface

Answer 42

Psoriasis

Question 43

lupus erythematoosus

Answer 43

-