Which does not have a positive Nikolsky sign?

A. Bullous Pemphigoid

B. Lichen Planus

C. Pemphigus

D. Benign Mucous Membrane (Cicatricial) Pemphigoid

B. Lichen Planus

Nikolsky sign: the ability of the Dr. to produce a blister by rubbing clinically normal skin or mucosa

* Erosive Lichen Planus may test Nikolsky sign +

Which has intraepthithelial vesicles?

Benign Mucous Membrane (Cicatricial) Pemphigoid

Pemphigus Vulgaris

Pemphigus

(all pemphigus types)

- Pemphigoid has sub-epithelial vesicles

_____ ______ are retained in parakeratinized epithelium.

Pyknotic nuclei

______ are the predominant cell type in stratified squamous epithelium.

Keratinocytes

In keratinized areas of the oral mucosa especially subject to friction, ____ _____ are usually prominent.

rete ridges

Where is desmoglein 3 found?

In desmosomes, which are intracellular bridges connecting keratinocytes to each other in the stratum spinosum.

What are Birbeck granules?

Where are they found?

small vesicles with a bulbous extension (tennis racket or rod-shaped granules) that are found in Langerhans cells of the oral stratified squamous epithelium

_______ anchor keratinocytes to the basement membrane.

Hemidesmosomes

Defective cellular cohesion involving plectin, BPAG180 transmembrane protein and integrin α6β4 causes clefting in which category of Epidermolysis bullosa?

the Hemidesmosomal type

- these proteins are all found in the hemidesmosomal plaque anchoring keratinocytes to the basement membrane.

Which represents a proliferation of cells normally found at the site of the lesion?

A. hamartoma

B. choristoma

C. teratoma

A. hamartoma

- choristomas are normal cells in an abnormal location

- teratomas are representative of more than 1 germ cell layer

Which keratinized masticatory mucosa has a submucosa beneath it?

The lateral hard palate

In which parts of the oral mucosa is lamina propria found?

all of them

In which part of the hard palate might "salivary gland tumor" be part of a differential diagnosis?

In the posterior; there are no salivary glands in the anterior hard palate, just fat.

Autoantigens against desmoglein 3 are found in which mucocutaneous disease?

Autoimmune Pemphigus

Which variant of pemphigus has affected areas similar to lupus (butterfly rash, back)?

pemphigus erythematosus

Which does not appear in pemphigus vulgaris?

A. "tombstone" basal cell layer

B. flaccid bullae

C. scarring of ulcers

D. intraepithelial vesicles

C. scarring of ulcers

Pemphigus vulgaris has a 10% mortality rate, usually due to complications from which type of treatment?

systemic corticosteroids

Multiple vesicles or ulcerations that heal with scarring, except in oral cavity.

BMMP

benign mucous membrane pemphigoid

What diseases are each of the following herpes viruses

associated with:

Herpes simplex 1

Herpes simplex 2

HHV 3

HHV4

Herpes simplex 1- skin and  mucosa above waist

Herpes simplex 2 - genital herpes infections

HHV 3 - Varicell Zoster Virus; chicken pox & shingles

HHV4 - Epstein-Barr Virus;

hairy leukoplakia

infectious mononucleosis

African Burkitt's lymphoma

nasopharyngeal carcinoma

What diseases are each of the following herpes viruses associated with:

HHV5

HHV6

HHV7

HHV8

HHV5 - Cytomegalovirus

HHV6 - roseola infatum

HHV7 - human B lymphotropic virus

HHV8 - Kaposi's sarcoma

Acantholysis leads to Tzanck cells in Pemphigus vulgaris.

What other diseases produce epithelial acantholysis/ Tzanck cells, along with "Cowdry type A inclusions"?

Herpes Simplex, VZV, Herpes Zoster

- cowdry type A inclusions refers to the glassy, uniform appearance of nuclei that results from nuclear enlargement and chromatin clearing.

Herpes lesions also show multinucleated cells.

The most common cause of recurrent erythema multiforme

HSV-1 infection

Herpes Simplex infection of fingers - found in dentists before universal precautions.

Herpetic Witlow

Hodgkins lymphoma can produce cells with "owl eye nuclei". What other disease produces endothelial and salivary duct epithelial cells that display these nuclei?

Hint: it is a common infection, but asymptomatic in immunocompetent people

CMV

cytomegalovirus

These spots present at an early stage of Rubeola (measles); they are comprised of focal mucosal erythema with central blue-white papules.

Koplik spots

- represent area of superficial epithelial necrosis

- usually on buccal and labial mucosa

Forcheimer's sign are small dark red papules affecting the soft and hard palate in conjunction with skin lesions. In what disease are they found?

Rubella (German Measles)

- mild illness caused by togavirus

- can cause birth defects

Which type of pneumonia was at one time very rare, but is seen as an opportunistic infection in about half of all AIDS patients?

Pneumocystitis carinii pneumonia

The most common intraoral manifestation of HIV infection

Candidiasis

- includes thrush (pseudomembranous), angular cheilitis,

erythematous, hyperplastic patterns

- predictive of development of AIDS within 2 years

"strawberry tongue", where edematous and hyperemic fungiform papillae project through a white coating,

and "raspberry tongue", when coating is lost and papillae are exposed, are characteristic of what illness affecting mostly children?

Scarlet Fever

- S. pyogenes toxins cause vascular dilation & damage

- Scattered petechiae on soft palate, exudate on tonsils

Cellulitis is an acute edematous inflammatory reaction which is rapidly spreading. S. pyogenes produces enzymes that allow rapid spread through tissues.

Name two types of cellulitis & characterize each.

Ludwig's Angina

- submandibular, sublingual, lat. pharyngeal spaces

- can originate from periapical infection of lower teeth

- clinically, area is brawny hard, fever, malaise

Erysipelas

- sharply demarcated, often due to trauma/S.pyogenes

- edematous hot shiny plaque, orange peel sometimes

- butterfly pattern seen when malar/face involved

- fever chills, malaise

This fusospirochetal infection was known as "trench mouth" in WWI.

What are some clinical manifestations?

NUG - necrotizing ulcerative gingivostomatitis

Clinically:

- initially red & painful gingivae

- later punched out erosions of interdental papillae,

covered by grey necrotic pseudomembrane

- ulcerations may spread along gingiva

- characteristic odor

- fever and malaise may be present

*diagnosis made on clinical observations, not histo

True or False:

Deep pockets are a characteristic feature of NUP

(necrotizing ulcerative periodontitis)?

False.

Deep pockets are not seen because gingival necrosis coincides with alveolar bone loss.

Name 4 types of periodontal disease associated with HIV infection.

Linear gingival erythema

NUP

NUG

(both of these may progress to Necrotizing stomatitis)

In what stage of syphilis do oral gummas and syphilitic glossitis appear?

Describe each lesion.

Tertiary Syphilis

- oral gummas start as rubbery necrotic nodules, leading to ulceration, which may heal or go on to produce oral-nasal fistula

- syphilitic glossitis presents as smooth bald tongue with areas of leukoplakia, microscopically diffuse interstitial chronic inflammation with endarteritis obliterans & fibrosis of the parenchyma

Sulfur granules are firm yellow bacterial colonies of 1 to 4 mm, and are found in healthy as well as diseased mouths. When certain bacteria penetrate deeper tissues they cause this disease.

What's the disease and which bacteria are the usual culprit?

Actinomycosis, caused by Actinomyces israeli

Median rhomboid glossitis (central papillary atrophy of the tongue) is caused by what organism?

C. albicans

- it occurs alone or as part of chronic multifocal candidiasis

Strawberry gingivitis presents early in this disease, for which elevated pANCAs and cANCAs are diagnostic.

Wegener's granulomatosis

- strawberry gingivitis is erythematous, granular gingival hyperplasia usually on facial attached gingiva

Sarcoidosis is an idiopathic multisystem granulomatous disease. Dry cough and dyspnea are two of the clinical symptoms. It is characterized histologically by non-caseating granulomas.

What type of lesions are found in the oral cavity?

Nodular lesions of the mucosa (normal color or discolored)

&

Alveolar bone loss and tooth mobility

Eosinophilic Ulcer (traumatic granuloma) is most common on the tongue. It looks like, and should be differentiated, from what?

cancer

This reversible condition is a mucosal reaction to the heat of cigarette smoke.

It presents as red macules representing inflamed minor salivary ducts, then progresses to diffuse keratinization and white papules with red punctate centers.

Nicotine Stomatitis

Acquired Angioedema

- due to mast cell degranulation and histamine release

(allergic reaction), mediated by IgE

- sudden, swift onset of a soft painless edematous swelling affecting lips, face, or neck. Oral involvement of floor of mouth, lips, tongue, uvula...

- patient must be observed until resolution in 24-72 hours

What type of testing would provide a definitive diagnosis for sarcoidosis?

What is the prognosis?

a positive Gallium 67 scintiscan with elevated ACE

60% of cases undergo spontanous resolution within 2 years

Corticosteroid treatment is successful treatment in 20%

 Mortality rate is 4-10% due to respiratory or cardiac failure

(Fair?)

What type of allergic contact stomatitis results from prolonged or frequent contact with cinnamon food additives?

What are the clinical presentations?

cinnamon-induced contact stomatitis

- adherent white oblong patches on buccal mucosa, along occlusal plane, w/ or w/out ulceration

-  may be present on lateral tongue, spreading to dorsum

- PLASMA CELL GINGIVITIS: fiery red gingiva, sea of plasma cells if biopsied

What benign disorder of the buccal mucosa presents as grey-white, bilaterally symmetrical filmy or wrinkled surface that does not wipe off but is reduced by stretching the mucosa?

Leukoedema

Which blanches with pressure: a hemangioma or a telangiectasia?

Telangiectasias blanch with pressure

Hemangiomas do not

- Telangiectasias seen in Hereditary Hemorrhagic Telangiectasia (Osler-Rendu-Weber disease) and CREST syndrome

Which disorder does not usually affect Parotid glands?

A. Mococele

B. Bacterial Sialadenitis

C. Mumps

D. Sialadenosis

A. Mococele

aka Mucus-Escape (Extravasation Phenomenon)

- these affect the minor glands, usually in lower labial mucosa, and more often in young people

- usually fluctuant, well-circumscribed, sessile, dome-shaped, often blue

- represent fluid filled cavity surrounded by granulation tissue, from trauma to duct and saliva escape/pooling

Name the 6 categories of MUCOSAL FLAT LESIONS

A. Solitary Ulcers

B. Multiple Ulcers

C. White Plaques and Patches

D. Red Macules and Patches

E. Red and White Macules and Patches

F. Pigmented Macules and Patches

Name the 6 categories of MUCOSAL RAISED LESIONS

G. Normal Colored, well defined nodules

H. Discolored well defined nodules

I. Irregular ulcerated enlargements

J. Verrucous enlargements

K. Multiple enlargements

L. Generalized gingival enlargements

In micro-examination of what oral lesion would you see:

• microabscesses (Monroe abscesses)
• migrating neutrophils in upper epithelium
• hyperkeratosis

?

A Psoriaform Lesion

including:

Erythema Migrans (migratory stomatitis/geographic tongue)

&

Reiter's syndrome

SSA(Ro) and SSB(La) are elevated in which two autoimmune diseases?

How would you test for these anitgenic non-nuclear DNA components?

Sjogren's and SLE

Use indirect immunofluorescence (IIF) to test serum

In which disease is it imperative to refer patient to opthamologist: P. vulgaris or Cicatricial Pemphigoid?

Cicatricial Pemphigoid

- 2/3 of patients have ocular lesions --> symblepharon & ankyloblepharon

- eyes not as severely affected in Bullous Pemphigoid

Describe the histopathologic features of "Lichenoid" lesions

• hyperkeratosis corresponding to white areas of lesion
• epithelial atrophy
• effaced basal cell layer
• saw-tooth rete ridges
• colloid bodies
• broad, band-like lymphocytic infiltrate

What oral lesion characteristically presents as a red/white plaque or ulcerations with radiating/sunburst white striae?

Lupus Erythematosus

Which disease/oral lesion does not have destruction of the basement membrane as part of its histopathology?

A. Epidermolysis Bullosa acquisita

B. Lichen Planus

C. SLE

D. MMP

E. Morsicatio buccarum

F. Erythema multiforme

E. Morsicatio buccarum

(chronic cheek biting)

- this presents as frictional keratosis, similar to linea alba

Which multiple mucosal ulcer diseases are always Nikolsky sign positive?

Which are sometimes or rarely Nikolsky sign positive?

Always:                Pemphigus vulgaris

BMMP

Epidermolysis Bullosa

Sometimes:         Erosive Lichen Planus

Erythema Multiforme

Systemic Allergy

In what type of Epidermolysis Bullosa would you be most likely to find microstomia, ankyloglossia, and loss of function due to healing of bullae with scarring?

 dystrophic

- vesicles and bullae result from minor trauma

- recessive form may be fatal

- clefting below the lamina densa

The classic skin lesion of_________is the target or iris lesion. It consists of concentric erythematous rings separated by rings of near-normal color. Typically, the extremities are involved, usually in a symmetric distribution.

Erythema Multiforme

- typically affects young adults

- prodromal symptoms before onset of lesions

- self-limiting but may recur

- minor form usually triggered by HSV

- Stevens-Johnson more severe, often triggered by drugs

- multiple oral ulcers and/or skin lesions

Name the 4 most common diseases responsible for

Desquamative Gingivitis

Mucous Membrane Pemphigoid

Erosive Lichen Planus

Pemphigus Vulgaris

Allergic Reactions

List 3 Oral White Plaques or Patches that normally appear bilaterally on the buccal mucosa.

Leukoedema

Lichen Planus

White Sponge Nevus

possibly also:              frictional keratosis

leukoplakia

True or False:

Untreated Pemphigus Vulgaris is potentially fatal.

True

- treat with steroid sparing immunosuppressives & systemic corticosteroids

- 10% mortality rate with treatment

Which two bilateral white lesions primarily affecting  the buccal mucosa demonstrate vacuolization of cells in the spinous layer?

Leukoedema

&

White sponge nevus

You would be most likely to find:

• widening of the PDL
• microstomia
• gingival recession and loss of attached gingiva
• partial mandibular resorption
• hypomobile tongue
• dysphagia
• xerostomia and cervical caries

in this immunopathologic condition

Scleroderma

aka Systemic sclerosis

- abundant collagen deposits throughout the body

- skin often affected first

- mouse or birdlike facies

- coup de sabre scarring

- sclerodactyly and Raynaud's

- pulmonary fibrosis common cause of death

- often accompanied by other automimmune diseases

"Anti-Smith" antibiodies are an immunologic finding that is virtually pathognomonic for what disease?

Systemic Lupus Erythematosus

- diagnostic criteria is based on the presence of at least 2 clinical and at least 2 abnormal immunologic findings

i.e. positive "lupus band test", ANAs (SSA-Ro)

- treatment with systemic corticosteroids, antimalarials, NSAIDS

- 90% 5 year, 80% 10 year, mostly due to kidney failure

CREST syndrome is thought to be a mild form of scleroderma. What does the acronym stand for?

C: calcinosis cutis - asympotomatic subcutaneous nodules

R: Raynaud's phenomenon

E: Esophogeal disfunction

S: sclerodactyly

T: telangiectasia - often on face, causes bleeding

What is the treatment for Herpes Simplex infections, or for Erythema Multiforme triggered by HSV infection?

acyclovir

Coxsackie Virus infections are usually seen in children.

Which of the 3 significant diseases caused by this virus usually has:

- no prodromal symptoms (but flu-like symptoms once lesions erupt)

&

- vesicles/ulcers that affect the tongue as well as buccal and labial mucosa

?

Hand, Foot and Mouth Disease

- mainly caused by virus A16

- affects more anterior parts of mouth than other 2

(Herpangina & Acute lymphonodular pharyngitis)

- variable numbers of cutaneous lesions appear on hands and feet

There is no loss of attachment and alveolar bone, but fever and malaise may be present in this oral "fusospirochetal" infection.

NUG

- diagnosis made on clinical data

- initially red and painful gingiva

- later punched-out erosions of interdental papilla

"Snail track" ulcers are confluent mucous patches.

In which stage of syphilis would you find these lesions?

What other lesions would you find in this stage?

Secondary Syphilis

- papules/split papules

- condyloma lata ("flat knuckle") papules

Name two types of Coxsackie virus infections that usually include a sore throat and lesions of the soft palate and/or tonsillar pillars.

Herpangina

&

Acute lymphonodular pharyngitis

(hand, foot, and mouth has no prodromal symtoms and affects anterior oral tissues)

Give the more common name for each disease:

Scleroderma

Cancrum oris

Hansen's Disease

Stomatitis venenata

Darier's Disease

Cannon's Disease

Von Recklinghausen's Disease

Sutton's Disease

Scleroderma aka Systemic sclerosis

Cancrum oris aka NOMA or Necrotizing stomatitis

Hansen's Disease aka Leprosy

Stomatitis venenata aka Allergic contact stomatitis

Darier's Disease aka Keratosis Follicularis

Cannon's Disease aka White sponge nevus

Von Recklinghausesn's Disease aka Neurofibromatosis 1

Sutton's Disease aka Major apthous stomatitis

Match the genetic marker to its syndrome:

HLA B27

HLA p51

HLA B27 - Reiter's syndrome

HLA p51 - Behcet's syndrome

Which would you be more likley to find on the lower lip:

Mococele /mucus-escape phenomenon

or

Mucus retention cyst

?

Mococele

- occur at any site where there are minor glands,

lower labial mucosa being most common location

- biopsy reveals fluid-filled cavity surrounded by granulation tissue w/ inflammatory cells including foamy macrophages

When edges of upper and lower eyelid grow fibrous tissue and fuse together it is called ________.

Ankyloblepharon

A raised, submucosal hemorrhage is called a _______.

An large non-raised, submucosal hemorrhage is a ____.

raised - hematoma

large (> 2 cm) non-raised - ecchymosis

True or False:

In acute bacterial sialadenitis biopsy should be avoided.

True

- biopsy may allow infection to spread beyond the infected parotid gland

- sialography also contraindicated in acute sialadenitis

Chronic sclerosing sialadenitis of the submandibular gland may cause what formation that is clinically difficult to distinguish from neoplasia?

Kuttner tumor

What causes chronic sclerosing sialadenitis?

How is it characterized histologically?

long-term obstruction of the salivary gland without infection

- characterized by acinar atrophy, chronic inflammation and marked fibrosis --> loss of function

- occasionally entire gland becomes calcified

Which is not a histological finding in SLE?

A. eosinophils

B. basal cell liquefaction

C. dense collagen deposition

D. perivascular lymphocytic infiltrate

E. subepithelial chronic inflammatory infiltrate

C. dense collagen deposition

- characteristic of systemic sclerosis

What disease am I?

- fissured tongue

- facial paralysis

- chronic, firm lip swelling

Melkerson-Rosenthal syndrome

Scrofula

 "tuberculosis of the neck"

mycobacterial lympadenopathy of oropharyngeal and cervical nodes

nodes may caseate with sinus tracts

A patient presenting with at least two of the following symptoms is diagnostic for this idiopathic multisystem disease:

recurrent genital ulcers

retinal vasculitis

erythema nodosum-like lesions

skin pathergy

What would be their oral manifestations?

Behcet's syndrome

 - oral apthous like ulerations (3x in 1 year)

Name 2 steroid-sparing agents that might be alternated with systemic corticosteroid therapy.

immuran & cyclosporine

Cheilitis granulomatosa is a component of Melkerson-Rosenthal syndrome. It presents clinically as firm swelling of the lip(s). What does it look like histologically?

typical granuloma morphology:

poorly-formed granulomas consisting of

- epithelioid cells

- multinucleate giant cells

- lymphocytes & macrophages

* no microorganisms or foreign material

Traumatic implantation of dental materials into the connective tissue below the sulcular epithelium would result in what type of forein body reaction?

Granulomatous gingivitis

- redness, pain, or sensitivity in involved area

- granulomatous inflammation

treatment is surgery

Which part of the body is not typically affected by the necrotizing vasculitis of Wegener's granulomatosis?

kidney

heart

lungs

ears

nose

heart

- upper & lower resp. tract typically involved (ears, nose, throat, lungs)

- renal necrotizing glomerulonephritis not uncommon - renal failure often cause of death

Anergy is a term in immunobiology that describes a lack of reaction by the body's defense mechanisms to foreign substances. Anergy is seen in most patients with this multisystem idiopathic disease.

HINT:  Heerfordt's syndrome and Lofgren's syndrome are also associated with this disease

Sarcoidosis

- HLA DR types may be susceptible

Erythema nodosum are red tender nodules that tend to itch and burn and are found on the legs. Name two diseases in which this may be found.

Behcet's syndrome

Sarcoidosis

You see a microscopic tissue sample that included Schaumann bodies (laminated basophilic calcifications) and asteroid bodies (stellate cellular inclusions).

What tissue should you biopsy?

What skin test could you expect to test positive?

What would you expect to see on their chest film?

biopsy superficial lobe of parotid gland

(93% success rate for Sarcoidosis)

Positive Kveim skin test

(4-6 weeks, 50-85% accuracy)

Chest film would show hilar lymphadenopathy

True or False:

Oral lesions may precede cutaneous lesions in both Pemphigus vulgaris and Crohn's disease.

True

Which is not a mucosal reaction to smoking?

A. Smoker's Melanosis

B. Stomatitis Venenata

C. Nicotine Stomatitis

D. Hyperkeratosis

B. Stomatitis Venenata

aka allergic contact stomatitis

- reverse smokers show pigmentation of the hard palate in smoker's melanosis

- palate also involved in nicotine stomatitis

Air introduced into a surgical flap or entrapped in a root canal during a dental procedure may lead to what type of mucosal reaction?

Cervicofacial emphysema

- crepitus and/or pain present

- resolves by itself in 4-7 days, prescribe antibiotics

Which is more common: allergic contact dermatitis or allergic contact stomatitis?

Allergic contact dermatitis

What do the following drugs all have in common?

antimalarials

AZT

minocycline

phenolphthalein

oral contraceptives

phenytoin

antihistamines

They produce oral pigmentation!

What disease am I?

defective desmosomes

skin papules

acantholytic dyskeratosis

Corps ronds

Keratosis Follicularis (Darier's Disease)

defective Ca pump gene: ATP2A2 on 12q23-24.1

treated with Vitamin A derivatives

Which disease am I?

Multiple hamartoma & neoplasia syndrome

PTEN mutation

multiple facial tricholemmomas

multiple oral papules (cobblestone mucosa)

acral keratosis (warty growth of hands)

Cowden Syndrome

Which has the worst prognosis?

Von Recklinghausen's Disease

Junctional Epidermolysis Bullosa

Pemphigus Vulgaris

Von Recklinghausen's Disease

aka

Neurofibromatosis Type 1

- 5 year survival rate only 15%

- dystrophic type of Epidermolysis Bullosa can be fatal

Mutations in collagen type VII cause which type of Epidermolysis Bullosa?

Which type is caused by mutations in the subunits for laminin?

Where does clefting occcur in each?

collagen type VII mutation -> clefting below lamina densa

= Dystrophic types

recessive dystrophic can be fatal

laminin subunit mutation -> clefting at lamina lucida

= Junctional type

dental abnormalities

Patients with Hurler's syndrome die from what by the age of 10?

cardiac disease

- accumulation of dermatan and heparan sulfates due to alpha-L-iduronidase deficiency

- oral manifestations: macroglossia, open mouth, thick lips, lucencies around developing teeth, slow eruption, gingival hyperplasia

True or False:

Salivary sialoliths are most common in older adults.

False.

They are most common in young and middle-aged adults

"The roof of my mouth suddenly fell out!"

- don't worry, it's benign

Necrotizing Sialometaplasia