Ch 16

Question 1

Ectodermal dysplasia

Answer 1

Ectodermal derived structure fail to develop.
x-linked, Male predominance.
reduced number of teeth with abnormal crowns.

Question 2

White Sponge Nevus/Genodermatosis

Answer 2

AD, benign skin disorder due to defects in Keratins 4, 13
Symmetrical, thickened, white plaques.
bilateral buccal mucosa.

Question 3

Peutz-Jeghers Syndrome

Answer 3

AD,
Freckle like lesion on hands, perioral skin and mucusa.
Intestinal polyposis; not premalignant.
GI obstructions due to intussusception.
Cancer predisposition. GI adenocarcinoma + others 18X

Question 4

Tuberous Sclerosis

Answer 4

Mental retardation
Angiofibromas of skin, fungal fibromas, rhabdomyoma.
Pitted anterior teeth and multiple fibrous papules
Shagreen patches; connective tissue hamartomas
Ash-leaf spots; ovoid areas of hypo pigmentation

Question 5

Pemphigus

Answer 5

Four related diseases: vulgaris, vegetans, erythematous, foliates.
Oral lesions first to show last to go.
Antibodies directed agains desmosomes= intraepithelial split.
M=F>50yo
Positive Nikolsky sign; bulla can be induced with lateral pressure.

Question 6

Pemphigus Vulgaris is associated with what disease?

Answer 6

Hailey-Hailey disease

Question 7

Mucous membrane pemphigoid/cicatricial pemphigoid

Answer 7

Twice as common as pemphigus
2:1 F:M >55
Noticeable intraoral blisters that do not scar.
OCULAR INVOLVEMENT; symblepharons, entropion leading to blindness.
Refer to ophthalmologist…corticosteroids.

Question 8

Erythema multiforme

Answer 8

50% can be traced to infection or medication.
Steven’s-Johnson syndrome; erythema multiform major
Lyell’s disease= toxic epidermal necrolysis.
Acute onset,
Young adults M>F
Prodromal stage; 1 wk prior; fever, headache, cough, sore throat.
Bull’s eye skin lesion.
hemorrhagic crusting of vermillion zone.
Discountinue medication.