Dermatologic Disease

Question 1

What group of inherited conditions has two or more ectodermal derived anatomic structures fail, typically including Skin, hair, nails, teeth, sweat glands?

Answer 1

Ectodermal Dysplasia

Question 2

Which best known ectodermal dysplasia is x-linked with Male predominance, and is characterized by heat intolerance, fine, sparse hair, periocular wrinkling with hyper pigmentation and fewer, conical shapped teeth?

Answer 2

Hypohidrotic Ectodermal Dysplasia

Question 3

A genetically determined skin disorder is called a?

Answer 3

Genodermatosis

Question 4

What AD lesion in keratin appears at birth or early childhood as a symmetrical thick, white, digress plaque on the bilateral buccal mucosa?

Answer 4

White sponge Nevus

*bening condition with no treatment

Question 5

What rare condition is characterized by Freckle-like lesions of the hands, perioral skin, and oral mucosa (lips especially), Intestinal polyposis
and a predisposition for affected patients to develop cancer?

Answer 5

Peutz-Jeghers Syndrome

*intestinal polyps are not premalignant

Question 6

Peutz-Jeghers syndrom patients are how many times more likely to have malignancy in their lifetime?

Answer 6

18x

*GI adenocarcinoma or other tumors such as breast cancer

Question 7

What condition is characterized by CNS manifestations, Angiofibromas of the skin, Ungual (or perungual) fibromas (fingernails), and Characteristic skin lesions?

Answer 7

Tuburous Sclerosis

Question 8

What are the 3 CNS manifestations of Tuburous Sclerosis?

Answer 8

1-Mental retardation
2-Seizure disorders
3-Potato-like growths (“tubers”)

Question 9

What are the two characteristic skin lesions of Tuburous Sclerosis?

Answer 9

1-Shagreen Patches (connective tissue hamartomas)

2-Ash-leaf spots (ovoid areas of hypo pigmentation)

Question 10

What condition has oral lesions that are the first to show but last to go?

Answer 10

Pemphigus Vulgaris

Question 11

The intraepithelial split that takes place in Pemphigus vulgaris is due to antibodies directed at what?

Answer 11

Desmosomes

Question 12

A bulla being induced with lateral pressure on normal appearing skin is called what?

Answer 12

Positive Nikolsky sign

*characteristic of pemphigus vulgaris

Question 13

Localized pemphigus in a small 1 cm or less usually on the palate is called?

Answer 13

Hailey Hailey Disease

Question 14

AKA Cicatricial pemphigoid, what condition is characterized by an intraoral blood blister and is twice as common as pemphigus?

Answer 14

Mucous Membrane Pemphigoid

Question 15

What are the two stages of occular involvement in mucous membrane pemphigoid?

Answer 15

1-Symblepharons (Scarring adhesions resulting from inflamed conjunctiva)
2-Entropion (eyelid turns inward)

Question 16

A subepithelial split is observed in what condition where antibodies attack the basement membrane?

Answer 16

Mucous Membrane Pemphigoid

Question 17

Which blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis is usually due to a preceding infection or exposure to medication?

Answer 17

Erythema Multiforme

*acute onset with a spectrum of clinical disease. Prodromal symptoms.

Question 18

When erythema multiforme is caused by infection it is usually _________ And when it is caused by medication it is usually _______

Answer 18

Herpes simplex

Antibiotics

Question 19

What characteristic erythematous lesions develop in Erythema Multiforme?

Answer 19

Target lesions

*also crusting of the vermilion zone of the lips

Question 20

Steven Johnson syndrome is usually triggered by a drug, involves skin, oral mucosa and either ocular or genital mucosa and is known as what?

Answer 20

Erythema Multiforme Major

Question 21

What is the most severe form of Erythema Multiforme?

Answer 21

Toxic Epidermal Necrolysis

Question 22

AKA geographic tongue or Benign Migratory glossitis, what condition has a serpentine border and can change from day to day?

Answer 22

Erythema Migrans

Question 23

What condition is characterized by a lace like network of white lines known as wickhams striae?

Answer 23

Lichen Planus

Question 24

If a lichen planus looking lesion is due to medication or amalgam it is known as?

Answer 24

Lichenoid mucositis

Question 25

What are the 4 Ps of Skin lesion in lichen planus?

Answer 25

1-Purple 2-Pruritus 3-Polygonal 4-Papules

Question 26

What are the two forms of lichen planus lesions?

Answer 26

1-Reticular (more common) | 2-Erosive (ulcerated)

Question 27

LIchen planus lesions have saw toothed rate ridges and can be induced by what 4 things?

Answer 27

1-Stress 2-Trauma 3-Advil (any NSAID except acetaminophen) 4-Yeast

Question 28

What condition occurs in recipients of allogeneic bone marrow transplantations?

Answer 28

Graft-Versus-Host Disease

Question 29

Of those with acute GVHD, __% have oral involvement,and _____% of chronic do

Answer 29

50, 80 *Most have fine reticular network of striae that look like lichen planus

Question 30

What classic example of an immunologically mediated condition is the most common collagen vascular or connective tissue disease in the US?

Answer 30

Lupus Erythematous

Question 31

With a 10:1 female predilection, what are 5 common findings of Systemic Lupus Erythematous?

Answer 31

``` 1-Fever 2-Weight loss 3-Arthritis 4-Fatigue 5-Malar rash (butterfly rash) – 50% ```

Question 32

What is the most significant aspect of Systemic Lupus Erythematous?

Answer 32

Kidney complications or failure

Question 33

Pericarditis is the most common cardiac complication of Systemic Lupus Erythematous, what are the warty vegetations called that affect the heart valves in this condition?

Answer 33

Libman-Sacks Endocarditis

Question 34

What condition is a disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts?

Answer 34

Systemic Sclerosis

Question 35

A vasoconstrictive event triggered by emotional distress or exposure to cold is called?

Answer 35

Raynaud's Phenomenon

Question 36

How does the diffuse, hard texture and smooth surface of Systemic Sclerosis patient's skin affect their oral cavity?

Answer 36

Results in microstomia making the lips hard to open and appearing to be puckered

Question 37

A mild variant of Systemic Sclerosis called localized scleroderma has what lesion?

Answer 37

Scar named en coup de saber ("strike of the sword")

Question 38

What 5 things does CREST syndrome have?

Answer 38

``` C – Calcinosis cutis R – Raynaud’s phenomenon E – Esophageal dysfunction S – Sclerodactyly T – Telangiectasia ``` *mostly women in their 60s

Question 39

The multiple, movable, nontender, subcutaneous, nodular calcifications in Crest disease are called?

Answer 39

Calcinosis Cutis

Question 40

The abnormal deposition of esophageal submucosa results in what?

Answer 40

Esophageal Dysfunction

Question 41

Stiff fingers that undergo permanent flexure resembling a claw are called?

Answer 41

Sclerodactyly

Question 42

Numerous red papillose that are 1-2 mm in size, most frequently on the vermillion zone that blanch are called?

Answer 42

Telangiectasias

Question 43

What condition is characterized by the development of a velvety, brownish alteration of the skin which can be a sign of internal cancer?

Answer 43

Acanthosis Nigricans *most are benign

Question 44

What are 5 possible causes of Benign Acanthosis Nigricans?

Answer 44

1-Obesity (termed pseudoacanthosis nigricans) 2-Inheritance 3-Various endocrinopathies (like diabetes mellitus) 4-Various syndromes (like Crouzon) 5-Various drugs (like oral contraceptives)