Oral Pathology Final Dermatologic Diseases

Front 1

Which of the following is a characteristic of Peutz-Jeghers syndrome?
a) Pigmental macules of the perioral skin and oral mucosa
b) Bilateral whitening of the buccal mucosa
c) Multiple papillary tumors of the tongue
d) Maxillary and mandibular tori
e) Multiple basal cell carcinomas of the face

Back 1

Pigmented macules of the perioral skin and oral mucosa

Front 2

Which of the following is true of white sponge nevus?
a) It is an autosomal dominant disease entity
b) The lesions are present at birth or by puberty
c) Lesions often appear as bilateral white lesions of the buccal mucosa
d) All of the above
e) A & C

Back 2

all of the above

Front 3

Specific disease entities associated with (clinical) desquamative gingivitis include which of the following?
a) Cicatricial pemphigoid
b) Lichen planus
c) Pemphigus vulgaris
d) all of the above
e) A & C

Back 3

All of the above

Front 4

Which of the following is most closely associated with lichen planus?
a) Butterfly rash
b) Target lesion
c) Wickham striae
d) Tzanck cells
e) Teeth floating in air

Back 4

Wickham striae

Front 5

Which of the following statements is true of cicatricial (benign mucous membrane) pemphigoid?
a) while the etiology is unknown, the disease is known to have an autoimmune component
b) Skin lesions are common occurring in approximately 75% of the cases
c) Immunofluorescence is an important and useful diagnostic technique
d) all of the above
e) A & C only

Back 5

A & C only
Skin Lesions are rare
Pemphigus has skin lesions more commonly

Front 6

Which of the following is a clinical manifestation associated with erythema multiforme?
a) Wickham striae
b) Butterfly rash
c) Target lesion
d) Facial paralysis
e) Epistaxis

Back 6

Target lesion

Front 7

Which of the following statements is true of pemphigus vulgaris?
a) Skin lesions rarely occur if at all
b) Most patients are under 20 years of age and are males
c) Oral lesions
d) Perilesional tissue characteristically shows subepithelial clefting (separation)
e) Most patients present with hypermobility of their joints

Back 7

Oral lesions may precede skin lesions and are often persistent thus are described as the first to show and last to go

Front 8

The term cicatricial indicates which of the following?
a) Areas of depigmentation
b) Scarring
c) Vesicle formation
d) Telangiectasia
e) Pruritus

Back 8

Scarring

Front 9

Which of the following statements is most accurate regarding the malignant transformation potential of lichen planus?
a) The potential is small and generally confined to cases of the erosive form of the disease
b) The potential is large and generally confined to cases of the bullous form of the disease
c) The potential is small and generally confined to cases of the reticular form of the disease
d) The potential is large and generally confined to cases of the reticular form of the disease
e) The potential is equal to that of proliferative verrucous leukoplakia and erythroplakia

Back 9

The potential is small and generall confined to cases of the erosive form of the disease

Front 10

This patient indicated that colon cancer ran in her family. This coupled with the radiopaque lesions observed on her panorex suggests which of the following?
a) McCune Albright syndrome
b) Gardner syndrome
c) Plummer Vinson syndrome
d) Von Recklinghausen disease
e) Sturge Weber syndrome

Back 10

Gardner Syndrome
Osteomas, Supernumerary teeth, Adenomatous colonic polyps > colon cancer

Front 11

The pigmented lesion of this patients face along with the unilateral, expansile lesion noted in the projected radiograph are most consistent with which of the following?
a) Peutz Jeghers syndrome
b) Sturge Weber syndrome
c) Neurofibromatosis I syndrome
d) Addison disease
e) McCune-Albright or Jaffe-Liechtenstein syndrome

Back 11

McCune Albright or Jaffe Liechtenstein syndrome

Front 12

This patient was a 65 year old male, who had hearing and visual difficulties. This information coupled with the two projected radiographs are most consistent with which of the following disease entities?
a) Gardner syndrome
b) Osteitis deformans
c) Von Recklinghausen disease
d) McCune-Albright syndrome
e) Nevoid basal cell carcinoma syndrome

Back 12

Osteitis Deformans

Front 13

The clinical and radiographic appearances of this middle aged male are suggestive of which of the following conditions?
a) Addison disease
b) Peutz-Jeghers syndrome
c) Hereditary hemorrhagic telangiectasia
d) Hereditary hemolytic anemia
e) Encephalotrigeminal angiomatosis

Back 13

Peutz-Jeghers Syndrome

Front 14

This 27 year old man complained of swelling, bone pain and looseness of his teeth of less than 5 months duration. Based upon the clinical history and the projected radiograph, this patient should be evaluated for which of the following conditions?
a) Fibrous dysplasia
b) Osteitis deformans
c) Osteosarcoma
d) Squamous cell carcinoma
e) Multiple myeloma

Back 14

Osteosarcoma
Widening of PDL, spiking of roots, osteophytic rxn
Mean age of 33 for Jaw

Front 15

A 12 year old boy is seen in consultation because of bilateral mandibular swelling. Radiographs show bilateral multilocular lesion in the ascending mandibular rami. The mother of this patient had similar findings as a child. The most likely diagnosis is which of the following?
a) Ellis-van Creveld syndrome
b) Nevoid basal cell carcinoma syndrome
c) Cleidocranial dysplasia
d) Cherubism
e) Gardner syndrome

Back 15

Cherubism

Front 16

The major concern for a dentist when treating a patient with Sturge- Weber syndrome is which of the following?
a) Severe infections
b) Spontaneous ulcerations
c) Gingival hemorrhage
d) Epithelial desquamation
e) The need for premedication

Back 16

???
spontaneous ulcerations

Front 17

Which of the following disease entities may commonly manifest a positive Nikolsky sign?
a) Pemphigus vulgaris
b) Lichen planus, reticular type
c) Cicatricial pemphigoid
d) All of the above
e) A & C only

Back 17

A &C only

Front 18

All of the following are true of lichen planus except?
a) Patients may have purple skin lesions that itch
b) If a malignant potential exists the risk is low
c) The keratotic white striations of this disease are known as Wickham striae
d) Most patients are middle-aged (or older) adults
e) The reticular form is usually symptomatic

Back 18

The reticular form is usually symptomatic

Front 19

Which of the following is true of lichen planus?
a) The reticular form is usually painful
b) Lesions frequently show light to dark brown pigmentation
c) A lace-like pattern of white striations is usually present in all forms
d) Is known to be caused by Epstein-Barr virus
e) Is most common in younger males

Back 19

A lace-like pattern of white striations is usually present in all forms

Front 20

Which of the following is the most common symptom of lichen planus involving the skin?
a) Pain
b) Itching
c) Crusting and oozing
d) Blister formation
e) Bleeding

Back 20

Itching
Note: Pruritic means itch so, pruritic, purple, polygonyl papules with a fine lace-like network of white lines

Front 21

Which of the following is true of pemphigus vulgaris?
a) Intraoral lesions generally precede skin lesions
b) No treatment necessary
c) Is associated with a positive Nikolsky sign (test)
d) All of the above
e) A & C

Back 21

A & C only
Treated w/systemic corticosteroids & immunosuppressive drugs

Front 22

All of the following may be true of benign mucous membrane (cicatricial) Pemphigoid except?
a) Intra-epithelial clefting is often seen
b) Corticosteroids are the drugs of choice for treatment
c) Patient should be sent to ophthalmologist to prevent ocular scarring
d) May involve eyes, genitalia and oral mucosa
e) Evidence suggests it is an autoimmune disease

Back 22

Intra-epithelial clefting is commonly seen
(this acantholysis is more commonly seen on Darier's Disease & Pemphigus)

Front 23

A patient with painful, peeling gingiva may have which ofthe following disease entities?
a) Erosive lichen planus
b) Pemphigus vulgaris
c) Cicatricial pemphigoid
d) All of the above
e) A & C only

Back 23

All of the above

Front 24

All of the following soft tissue lesions would have a female gender predilection except. Which one is the exception?
a) Lichen planus
b) Cicatricial pemphigoid
c) Squamous cell carcinoma
d) Desquamative gingivitis
e) Salivary gland tumors

Back 24

Squamous cell carcinoma

Front 25

What is a group of inherited conditions in which two or more ectodermally derived structures fail to develop, Aplasia of the skin, nails, teeth and sweat glands can occur?

Back 25

Ectodermal Dysplasia

Front 26

Which type of ectodermal dysplasia is X-linked (male), Heat intolerance due to lack of Sweat Glands, fever, Sparse, fine, blond hair, Xerostomia due to lack of salivary glands

Back 26

Hypohidrotic Type of Ectodermal Dysplasia

Front 27

What is an autosomal dominant skin disorder with a high degree of penetrance, present at birth or by adolescence, condition due to a defect in the normal keratinization of the mucosa?

Back 27

White sponge Nevus

Front 28

What are the clinical features of White Sponge Nevus?

Back 28

Symmetric, thickend, white corrugated/velvety plaque
Buccal mucosa mostly (may affect tongue, alveolar ridge or FOM)
Nasal, esophageal, & anogenital membranes may be involved
Patients asymptomatic

Front 29

What condition has prominent Hyperparakeratosis & acanthosis with clearing of cytoplasm of cells in spinous layer, similar to leukoedema & HBID, Eosinophilic Condensation of Jeratin Tonofilaments in Perinuclear Region?

Back 29

White sponge Nevus

Front 30

What condition is autosomal dominant, mutated STK11 gene, Characterized by freckle-like lesions of hands, perioral, perinasal skin & Oral Mucosa, also Intestinal Polyposis?

Back 30

Peutz-Jeghers Syndrom

Front 31

What are the clinical features of Peutz Jeghers syndrome?

Back 31

Skin lesions
Intestinal Polyps
2-3% develop GI adenocarcinomas
Oral lesions appear as 1-4 mm Blue-Gray-Brown macules affecting labial/buccal mucosa and tongue in approximately 90% of Patients

Front 32

What condition is a group of at least 10 Inherited connective tissue disorders attributed to abnormal collagen formation. Patients usually exhibit Hypermobility of joints, are Easily Bruised, Elasticity of the Skin?

Back 32

Ehlers-Danlos Syndromes

Front 33

What are the clinical features of Ehlers-Danlos Syndromes?

Back 33

50% of the patients can touch tip of nose with their tongue (Gorlin Sign)
Minor manipulations of oral tissue may cause bruising and/or bleeding
TMJ subluxation
Tooth abnormalities

Front 34

Direct Immunofluorescence as shown on the left is used to detect?

Back 34

Pemphigoid
The area lit up is the basal cell layer
In pemphigoid you get a Subepithelial blister

Front 35

Indirect Immunofluorescence shown on the right is used to detect?

Back 35

Pemphigus
The area lit up is between the epithelial cells
Remember that with pemphigus, we get a blister within the epithelium

Front 36

Pemphigoid is detected using direct immunofluorescence to detect?

Back 36

Hemidesmosomes

Front 37

Pemphigus uses Indirect Immunofluorescence to detect?

Back 37

Desmoglein 3 or dosmosome

Front 38

What are the 4 autoimmune diseases of Pemphigus?

Back 38

P vulgaris
P vegetans
P erythematosus
P foliaceus

Front 39

What condition has oral lesions that appear as the first sign, giving them the description "first to show and last to go", blistering is a result of autoantibodies directed against epidermal cell surface glycoprotein, a component of Desmosomes?

Back 39

Pemphigus Vulgaris

Front 40

What are the clinical features of Pemphigus Vulgaris?

Back 40

50 years
Vesicles, ulcerations, and erosions
Painful
Vesicle rupture quickly, so clinicians do not see these as often
Positive Nikolsky Sign - push on normal appearing tissue and place lateral pressure, you will get a bullous or it will completely strip off

Front 41

T/F
You can have a positive nikolsky sign in both pemphigus and pemphigoid

Back 41

True

Front 42

When you take a biopsy to test for pemphigus, where should you take it from?

Back 42

Perilesional area

Front 43

How does Pemphigus appear histologically?

Back 43

The superficial layers of epithelium have been sloughed leaving only the basal cells which have been described as a row of tombstones
The separation of the individual cells is termed acantholysis
The loos cells tend to become rounded Tzanck cells (not patholognomonic for this lesion)
Direct immunofluorescence is helpful in the diagnosis

Front 44

What are the synonyms for Mucous Membrane Pemphigoid?

Back 44

Cicatricial pemphigoid
Benign mucous membrane pemphigoid

Front 45

What is a chronic blistering mucocutaneous autoimmune disease which clinically may resemble pemphigus, but this focus is on mucous membrane and will scar after healing?

Back 45

Cicatricial Pemphigoid

Front 46

What are the features of Cicatricial Pemphigoid?

Back 46

60 years olde 2:1 Female
Lesions on mucosa (skin lesions extremely rare)
Desquamative gingivitis

Front 47

What are the histologic features of Cicatricial pemphigoid?

Back 47

Subepithelial clefting
Direct immunofluorescence shows a linear band at basement membrane zone
Immunoreactants IgG & C3

Front 48

What are the 3 groups of erythema multiforme?

Back 48

Erythema Multiforme Minor
Erythema Multiforme Major
Toxic Ectodermal Ectolysis

Front 49

What condition has an oset with wide spectrum of presentations ranging from ulcerations affecting oral cavity to a severe form with sloughing and ulceration of the entire skin and mucosal surfaces (toxic epidermal necrolysis or Lyell's disease)?

Back 49

Erythema Multiforme

Front 50

What did Erythema Multiforme Major also go by?

Back 50

Stevens Johnson Syndrome

Front 51

What is a more severe form of Erythema Multiforme triggered by drugs. Occular & genital lesions must accompany oral and skin lesions to diagnose Stevens-Johnson Syndrome**test question

Back 51

Erythema Multiforme Major

Front 52

What is the Most severe form of Erythema Multiforme , has female predilection, skin lesion heal in 2-4 weaks if patient survives?

Back 52

Toxic Erythema Necrolysis

Front 53

What are the clinical features of erythema migrans?

Back 53

Erythema (red center) is due to atrophy of filiform papillae and may be bordered by a white line
Migratory
May have fissured tongue

Front 54

T/F
A variety of drugs are known to induce similar appearing lesions for which the term lichenoid mucositis or lichenoid drug reaction is used, but this is NOT the same as Lichen Planus

Back 54

True

Front 55

Who is most commonly affected by lichen planus?

Back 55

3:2 Female
Middle aged to older

Front 56

What do the skin lesions of Lichen Planus appear as?

Back 56

Pruitic, purple, polygonal papules with a fine, lace-like network of white lines known as Wickham's Striae

Front 57

What are the two types of Lichen Planus?

Back 57

Reticular Lichen Planus - majority, Wickham striae, asymptomatic occuring on buccal mucosa bilaterally

Erosive Lichen Planus - Ulcerations, Fine white radiating striae (keratotic areas) can be seen at edge of lesion, Desquamative Gingivitis

Front 58

What conditions result in desquamative gingivitis?

Back 58

EROSIVE Lichen Planus
Pemphigus vulgaris if it only involves the gingiva
Cicatricial pemphigoid if it only involves the gingiva

Front 59

Which type of Lichen Planus is the most common form, occuring on buccal mucosa bilaterally, Wickham's Striae?

Back 59

Reticular Lichen Planus

Front 60

Which type of Lichen Planus has Central areas of Ulceration, Fine white radiating striae (keratotic areas) at edge of lesion, Desquamative gingivitis?

Back 60

Erosive Lichen Planus

Front 61

What is the most common collagen vascular disease or connective tissue disease in the US?

Back 61

Lupus

Front 62

What are the clinical features of SLE?

Back 62

Females 10:1
31 years old
Feverm weight loss, arthritis & malaise
Butterfly rash
Kidney problems
Libman Sacks endocarditis
Lichenoid appearance

Front 63

What condition is charaterized by dense collagen deposits in tissues of the body?

Back 63

Systemic Sclerosis (scleroderma)

Front 64

What are the clinical features of systemic sclerosis?

Back 64

Insidious onset
3:1 Female
Raynaud's phenomenon is first sign
skin deelops a diffuse, hard, smooth texture
Purse string appearance of lips
Diffuse Widening of PDL is often seen radiographically

Front 65

What is CREST syndrome?

Back 65

Limited Scleroderma
Calcinosis cutis
Raynaud's phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasias