chpt 16- dermatology ppt Flashcards

1
Q

inherited defects of ectoderm ally derived anatomic structures

A

ectodermal dysplasia

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2
Q

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

A

ectodermal dysplasia

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3
Q

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

A

ectodermal dysplasia

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4
Q

tx for ectodermal dysplasia

A
  • genetic counseling

- prosthetic replacement of teeth

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5
Q

Why would an ectodermal dysplasia patient have xerostomia

A

decreased salivary gland development

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6
Q

Tooth problems with ectodermal dysplasia

A

hypodontia

Cone shaped teeth

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7
Q

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

A

White sponge nevus (Canon’s Disease)

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8
Q

Tx for white sponge nevus

A

none

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9
Q

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

A

Hereditary Benign Intraepithelial Dyskeratosis

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10
Q

Histology Dr Siversky said to know for Hereditary Benign Intraepithelial Dyskeratosis

A

Epithelial clefting

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11
Q

What 3 diseases show intra-epithelial clefting

A

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

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12
Q

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

A

Keratosis follicularis (Darier’s disease)

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13
Q

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

A

multiple white papules

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14
Q

How treat Keratosis Follicularis/ Darier’s Disease

A

Keratolytic agents or emollients

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15
Q

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

A

Warty dyskeratosis

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16
Q

Where does the single white papule of Warty dsykeratoma appear

A

hard palate or alveolar ridge

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17
Q

Tx for warty dyskeratosis

A

Excision

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18
Q

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

A

Peutz Jeghers

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19
Q

2 disorders that have intestinal polyposis

A

1) Peutz Jegers Syndrome

2) Gardner Syndrome

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20
Q

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

A

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

21
Q

Treatment for Peutz-Jeghers

A

Monitor for intussusceptions or malignant transformation

22
Q

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

A

Peutz Jegher

23
Q

what percent of Peutz Jegher vs Gardner intestinal polyposis become malignant

A

PG: 2-3%
Gardner: 100%

24
Q

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

A

Hereditary Hemorrhagic telangiectasia

25
Q

Another name for HHT

A

Osler-Weber-Rendu Syndrome

26
Q

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

A

Hereditary Hemorrhagic telangiectasia

27
Q

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

A

Ehler’s Danlos

28
Q

Oral manifestations of Ehlers-Danlos

A
  • subluxation of TMJ

- Don’t respond well to surgery due to defective collagen Bruise and bleed easily

29
Q

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

A

Lichen Planus

30
Q

Demographics for Lichen planus

A

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

31
Q

Is Lichen planus only caused by autoimmune response

A

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

32
Q

Oral manifestation of Lichen Planus

A

Striae of Wickham

33
Q

skin manifestations of Lichen planus

A

purple puritic polygonal papules

34
Q

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

A

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

35
Q

Histology for Lichen Planus

A

saw tooth rete pegs and loose basal cell layer

36
Q

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

A

Erosive. Corticosteroids because it is autoimmune mediated

37
Q

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

A

candidiasis

38
Q

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

A

Nikolsky sign

39
Q

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

A

Pemphigus

Pemphigoid

40
Q

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

A

Pemphigoid (goid goes down to the basement)

41
Q

Histologically, which has intraepithelial separation: Pemphigoid or pemphigous

A

Pemphigus

42
Q

Which has larger lesions Pemphigoid or pemphigous and why

A

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

43
Q

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

A

benign mucous membrane pemphigoid

44
Q

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

A

vesicles are less than 5mm

Bullae are greater than 5 mm

45
Q

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

A

subepithelial clefting Immunofluorescence at basement membrane

46
Q

Most commonly used antibiotics for treating benign mucous membrane pemphigoid

A

Tetracycline Niacinamide Nicotinamide

47
Q

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

A

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

48
Q

Most common of the autoimmune blistering conditions

A

bullous pemphigoid