chpt 16- dermatology ppt

Question 1

inherited defects of ectoderm ally derived anatomic structures

Answer 1

ectodermal dysplasia

Question 2

affects the skin, hair, nails, teeth, sweat glands

Answer 2

ectodermal dysplasia

Question 3

characterized by fine, sparse hair, hypodontia, heat intolerance, periocular hyper pigmentation, xerostomia

Answer 3

ectodermal dysplasia

Question 4

tx for ectodermal dysplasia

Answer 4

• genetic counseling

- prosthetic replacement of teeth

Question 5

Why would an ectodermal dysplasia patient have xerostomia

Answer 5

decreased salivary gland development

Question 6

Tooth problems with ectodermal dysplasia

Answer 6

hypodontia

Cone shaped teeth

Question 7

Benign, autosomal dominant inherited skin disorder manifesting as thick white buccal mucosa bilaterally, may be corrugated or velvety

Answer 7

White sponge nevus (Canon’s Disease)

Question 8

Tx for white sponge nevus

Answer 8

none

Question 9

Autosomal dominant condition seen in descendants of North Carolina Indians, with thick white plaques on the buccal mucosa(like white sponge nevus) and the conjunctiva

Answer 9

Hereditary Benign Intraepithelial Dyskeratosis

Question 10

Histology Dr Siversky said to know for Hereditary Benign Intraepithelial Dyskeratosis

Answer 10

Epithelial clefting

Question 11

What 3 diseases show intra-epithelial clefting

Answer 11

1) HBID
2) Darier’s Disease
3) Pemphigus Vulgaris

Question 12

Autosomal dominant skin problem with erythematous puritic papules on the trunk and scalp with FOUL ODOR, dystrophic nails (ridged and split)

Answer 12

Keratosis follicularis (Darier’s disease)

Question 13

What is the oral manifestations of Keratosis Follicularis/ Darier’s Disease

Answer 13

multiple white papules

Question 14

How treat Keratosis Follicularis/ Darier’s Disease

Answer 14

Keratolytic agents or emollients

Question 15

Histopathologically identical to Darier’s Disease , but manifests orally as a single white papule

Answer 15

Warty dyskeratosis

Question 16

Where does the single white papule of Warty dsykeratoma appear

Answer 16

hard palate or alveolar ridge

Question 17

Tx for warty dyskeratosis

Answer 17

Excision

Question 18

Autosomal dominant with freckle like lesions in and around oral cavity, on the hands, and having intestinal polyps with a predisposition to go to adenocarcinoma

Answer 18

Peutz Jeghers

Question 19

2 disorders that have intestinal polyposis

Answer 19

1) Peutz Jegers Syndrome

2) Gardner Syndrome

Question 20

What is the difference, as far as the intestinal polyps, between Peutz-Jeghers and Gardners Syndrome

Answer 20

Peutz-Jeghers has benign intestinal polyps while Garderns are going to go malignant

Question 21

Treatment for Peutz-Jeghers

Answer 21

Monitor for intussusceptions or malignant transformation

Question 22

characterized by brown/black freckle like lesions in and around the oral cavity –> perioral and intramural and circumoral pigmentation

Answer 22

Peutz Jegher

Question 23

what percent of Peutz Jegher vs Gardner intestinal polyposis become malignant

Answer 23

PG: 2-3%
Gardner: 100%

Question 24

Autosomal dominant conditions of multiple vascular hamartomas due to decreased blood vessel wall intregrity.

Answer 24

Hereditary Hemorrhagic telangiectasia

Question 25

Another name for HHT

Answer 25

Osler-Weber-Rendu Syndrome

Question 26

Associated with frequent epistaxis, telangiectasias that are intraoral, hands, feet, GI tract, GU tract and eye, includes iron deficiency anemia if severe

Answer 26

Hereditary Hemorrhagic telangiectasia

Question 27

Genetic abnormalities that lead to abnormal collagen manifesting as hypermobile joints, elasticity of skin

Answer 27

Ehler's Danlos

Question 28

Oral manifestations of Ehlers-Danlos

Answer 28

- subluxation of TMJ | - Don’t respond well to surgery due to defective collagen Bruise and bleed easily

Question 29

Chronic inflammatory disease causing white striations, papules or plaques on buccal mucosa, tongue and gingival, thought to be autoimmune related by CD8 T cells causing oral epithelial cells apoptosis

Answer 29

Lichen Planus

Question 30

Demographics for Lichen planus

Answer 30

45-60 y.o (rare in children) Women 2x more than men

Question 31

Is Lichen planus only caused by autoimmune response

Answer 31

No, there can be drugs whose side effects cause a Lichenoid drug reaction, however, that goes away when drugs are stopped. True Lichen planus is chronic

Question 32

Oral manifestation of Lichen Planus

Answer 32

Striae of Wickham

Question 33

skin manifestations of Lichen planus

Answer 33

purple puritic polygonal papules

Question 34

When biopsying Lichen planus, do you biopsy the red or the white area

Answer 34

White area, red will just show ulceration (basement membrane with no epithelial covering)

Question 35

Histology for Lichen Planus

Answer 35

saw tooth rete pegs and loose basal cell layer

Question 36

Which phase of Lichen planus is treated: Reticular or erosive, and with what

Answer 36

Erosive. Corticosteroids because it is autoimmune mediated

Question 37

What can superimpose on top of Lichen planus especially during corticosteroid treatment of the Erosive form

Answer 37

candidiasis

Question 38

Dislodgement of skin by lateral pressure, can be a symptom in multiple diseases

Answer 38

Nikolsky sign

Question 39

2 disease we have been taught that have a positive Nikolsky sign

Answer 39

Pemphigus | Pemphigoid

Question 40

Histologically, which has a change in the basement membrane: Pemphigoid or pemphigous

Answer 40

Pemphigoid (goid goes down to the basement)

Question 41

Histologically, which has intraepithelial separation: Pemphigoid or pemphigous

Answer 41

Pemphigus

Question 42

Which has larger lesions Pemphigoid or pemphigous and why

Answer 42

pemphigoid, because they are thicker (all the way to the basement)

Question 43

Pemphigoid type that affects women over the age of 60 manifesting orally as vesicles or bullae

Answer 43

benign mucous membrane pemphigoid

Question 44

What are the clinical sizes of the benign mucous membrane pemphigoid vesicles and bullae

Answer 44

vesicles are less than 5mm | Bullae are greater than 5 mm

Question 45

What is the histology for benign mucous membrane pemphigoid (Siversky says to be able to ID this for the fina

Answer 45

subepithelial clefting Immunofluorescence at basement membrane

Question 46

Most commonly used antibiotics for treating benign mucous membrane pemphigoid

Answer 46

Tetracycline Niacinamide Nicotinamide

Question 47

If going to treat someone with benign mucous membrane pemphigoid with Dapsone, what must you check first

Answer 47

that they are not Glucose-6-Phosphate dehydrogenase deficient. Can cause aplastic anemia

Question 48

Most common of the autoimmune blistering conditions

Answer 48

bullous pemphigoid