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Oral path test 4
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04/04/2009

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Term
What are the first and second most common sites for dentigerous cysts?
  
Definition
  mandibular third molar; maxillary canine
Term
What lesions can potentially arise from an epithelial cystic lining?
  
Definition
 ameloblastoma, squamous cell carcinoma, and muoceoepidermoid carcinoma
Term
What are the differences between the odontogenic keratocyst and the keratinizing odontogenic cyst?
Definition

OKC - epi thin lining 6-8 cells thick with prominent palisaded basal cell layer and a corrugated  parakeratin surface - High recurrence rate; highest of any jaw cyst

 

KOC - epi lining may be thin also but lacks a palisaded basal cell layer and has an orthokeratin surface - Low recurrence rate 

 

 

Term
What are the clinical signs associated with the nevoid basal cell carcinoma syndrome?
Definition
(Gorlin syndrome)
    autosomal dominant with high penetrance but variable expressitivity
Multiple basal cell carcinomas, multiple OKCs of the jaws, bifid ribs, scoliosis, calcification of falx cerebri, frontal bossing, ocular hypertelorism, spina bifida, palmer & planter skin pits w/ basal cell carcinoma at base of the pits
Term
Which odontogenic cysts only occur in the soft tissues?
   
Definition
Eruption cyst (hematoma) variant of dentigerous cyst; gingival cyst of the adult; dental lamina cysts
    of the newborn
Term
What is the most common jaw site for the lateral periodontal cyst?
   
Definition
Mandibular canine-premolar
Term
Which odontogenic cyst exhibits ghost cells?
Definition
Calcifying Odontogenic Cyst (Gorlin cyst)
    Ghost cell = loss of nuclear staining; become calcified with time
         Unilocular, mixed RL/RO (scattered calcifications)
         Two types: Type 1 (cystic) & Type 2 (solid = odontogenic ghost cell tumor
Term
What is marsupialization?
   
Definition
Drainage to reduce the size of large intrabony lesion prior to its definitive surgical procedure
Term
What are the 3 types of unicystic (unilocular) ameloblastoma and how does their treatment differ?
Definition
   Luminal – lining affected only
    Intraluminal – lining with proliferation into the lumen
    Mural –  within the CT wall and may spread beyond wall into surrounding bone trabeculae
    Tx  - enucleation and curettage w/ minimal local bone removal for luminal & intraluminal types but en bloc
        resection w/ surgical margin evaluation for mural type
Term
What are the gross, microscopic and radiographic features of an adenomatoid odontogenic cyst/tumor?
Definition
   associated with an impacted tooth, esp. the ant. maxilla (e.g. canine) of     teenage females; expansive but             painless
    Gross: very thick capsule
Radiographic: well circumscribed RL w/ increasing RO over time of scattered calcifications; surrounds     crown & portion of the root, adjacent roots may be displaced
Histo: duct-like appearing odontogenic structures, thick fibrous capsule, amyloid deposition rarely but not     as frequent as Pindborg tumor
Tx: enucleation only (b/c thick capsule); no recurrence
Term
What is the most common site for an ameloblastic fibro-odontoma?
Definition
 Posterior mandible
Term
What are the clinical features of a conventional (solid and multicystic cystic) ameloblastoma?
Definition
most often posterior mandible, may be assoc w/ unerupted tooth in equal gender incidence; slow expansile growth; tooth displacement or mobility; root resorption; may cross midline
Term
What disease is characterized by the sign of increased body temperature in correlation to rising ambient   temperature?    
  
Definition
 Hypohidrotic Ectodermal Dysplasia – due to lack of sweat glands
    Fever of unknown origin is 1st sign
Term
. What genetic disease shows the signs of sparse hair, absence of sweat glands and malformed or missing teeth?
Definition
    Hypohidrotic Ectodermal Dysplasia
Term
Where are oral lesions of white sponge nevus seen?

Definition
    bilateral, buccal mucosa is most common site - thick, white, folded - - but can be any oral
    mucosa site; also seen in nasal, esophageal, laryngeal, anogenital mucosa – NOT CONJUNCTIVA OF
    EYE
Term
What is the treatment for white sponge nevus?
  
Definition
 No tx needed; observation only; can be diagnosed with cytology rather than biopsy
Term
. What genodermatosis is characterized by corrugated white plaques on the oral mucosa and conjunctival mucosa?
Definition
   Hereditary Benign Intraepithelial Dyskeratosis (HBID)
Term
What disease is characterized by abnormal collagen production resulting in hypermobility of the joints and extreme elasticity of the skin?
Definition
  Ehlers-Danlos syndromes
Term
What is Gorlin's sign and what is it associated with?        
  
Definition
 Hypermotility of tongue seen in Ehlers-Danlos syndromes - - touch the tip of the nose with the tip of the
    tongue.
Term
What disease is characterized by the formation of cutaneous angiofibromas and mental retardation?    
  
Definition
 Tuberous sclerosis; genetic disease w/ shagreen patches, ash leaf spots, CNS hamartomas (“tubers”)
Term
What autoimmune disease attacks desmosomal attachments?
Definition
Pemphigus vulgaris – intraepithelial split; can be diagnosed with cytology
Term
What disease produces acantholytic, intraepithelial blisters?
   
Definition
Pemphigus vulgaris. Blisters (vesicles, bullae) b/c epithelial cells no longer bond to each other
    (acantholysis); can be preceded by lymphoma/leukemia
Term
 Blistering of non-lesional skin or mucosa from lateral pressure is known as?
Definition
Positive Nikolsky sign; seen in pemphigus vulgaris, benign mucous membrane pemphigoid (cicatricial pemphigoid)
Term
Which autoimmune disease shows positive indirect immunofluorescence in the majority of cases?
  
Definition
 Pemphigus vulgaris – direct immunofluorescence is also used.
    Indirect immunofluorescence = pt’s blood on monkey tissue to show antibodies in blood
    Both pemphigoid and erosive lichen planus require direct IF since there is not much circulating antibodies     in the blood to be detected by indirect IF.
Term
What chronic blistering disease exhibits sub¬epithelial separation with positive direct immunofluorescence at the basement membrane?
   
Definition
Benign mucous membrane pemphigoid
Term
What is a major complication associated with benign mucous membrane pemphigoid?
   
Definition
Involvement of the eye (scarring) which can lead to blindness
Term
.  What is the clinical term for superficial erosion of the attached gingiva (and nowhere else!) due to a blistering process?
 
Definition
  Desquamative gingivitis
Term
What blistering disease can cause scarring and fusion of the digits and may cause death at an early age?    
   
Definition
Epidermolysis Bullosa (dystrophic, recessive type)
    Fusion of digits = “mitten-like” deformity; may also have esophageal strictures
Term
. What tissue is primarily affected by discoid lupus erythematosus?
Definition
Skin -scaly patches made worse w/ sunlight that heal w/ scarring; may be hyper or hypo-pigmented
Term
What disorder has cutaneous lesions that are exacerbated by ultraviolet radiation?
  
Definition
 Chronic discoid lupus erythematosus
Term
What disease is associated with Liebman-Sachs endocarditis?
   
Definition
Systemic lupus erythematosus (SLE); heart valve damage is common.
Term
. What disease will show positive lab findings for serum rheumatoid factor, antinuclear antibodies and antibodies to double-stranded DNA?  What can oral form look like?
  
Definition
 Lupus erythematosus;  oral can look like erosive lichen planus
Term
. What condition is characterized by vasoconstriction of the extremities triggered by emotion or cold?
 
Definition
   Raynaud’s phenomenon seen in progressive systemic sclerosis (scleroderma)
Term
. What disease advances its course by deposition of dense collagen in tissues and organs?
  
Definition
Scleroderma (PSS); leads to mask-like facies, loss of ala of nose, and fibrosis of vital organs (fatal)
Term
. What disease produces diffuse, generalized widening of the periodontal ligaments seen on radiographic exam?
   
Definition
Scleroderma (PSS)
Term
What is the name for the localized variant of progressive systemic sclerosis?
  
Definition
 Morphea; when its a linear scar on the face that can extend into the mouth = coupe de sabre
Term
. What is the name for the lace-like lines seen with reticular lichen planus?
 
Definition
   Wickham’s striae
Term
What is the most common site for oral lichen planus to be seen?
 
Definition
  Buccal mucosa
Term
What are the different oral forms of lichen planus and where do they occur?
       
Definition
Reticular form - lace-like appearance; buccal mucosa most common, Coalescing papules form Wickham’s striae
        Erosive form - ulcer w/ erythematous border; buccal mucosa, gingiva, tongue
        Hyperplastic (plaque) form – dorsal tongue
        Bullous form – skin; uncommon in oral cavity
Term
Lymphatic destruction of the basal layer with vacuolopathy and liquifactive necrosis are histologic hallmarks of what disease?    
  
Definition
 Lichen planus
Term
What disease has cutaneous target lesions as a classic presentation?
   
Definition
Erythema multiforme  = blistering, ulcerative, mucocutaneous disease of skin; extreme form is Stevens-    Johnson syndrome with oral, skin, genital and eye involvement
Term
Erythematous, (flat lesions of the tongue from atrophy of the filiform papillae is pathognomonic for what disease?
   
Definition
Geographic tongue (aka benign migratory glossitis, erythema migrans)
Term
What is the classic triad of signs seen in Reiter's syndrome?
  
Definition
Non-gonococcal urethritis (first sign), arthritis, conjunctivitis
Term
What is the condition in which papillary oral and cutaneous lesions present as an early sign of internal malignancy?
 
Definition
  Acanthosis nigricans   
Term
What are the inheritance patterns of the various lipid reticuloendothelioses? Inheritance patterns of mucopolysaccharidoses?
   
Definition

Lipid Reticuloendotheliosis- Affected patients lack certain enzymes necessary for processing specific lipids

 

Gaucher’s (most common), Neiman-Pick, Tay-Sacks = autosomal recessive  
    

 

 Mucopolysaccharidosis- Lack of any one of several enzymes required to process glycosaminoglycans

 

Hurler, Scheie, Sanfilippo A, Morquio, Maroteaux Lamy  = autosomal recessive; Hunter = x-linked
    recessive

Term
. What causes Neiman-Pick disease?
   
Definition
Types A & B - deficiency of acid sphingomyelinase causes accumulation of sphingomyelin in macrophage
    lysosomes.
     Type C - NPC-1 gene mutation (cholesterol processing)
Term
What are the oral manifestations of jaundice?
 
Definition
  Yellow discoloration of mucosa
Term
What is the ultrastructural appearance of amyloid?
 
Definition
  Beta-pleated sheet pattern
Term
What is the characteristic appearance of the red blood cells in pernicious anemia?
   
Definition
megaloblastic (enlarged), hyperchromic
Term
What serum levels are elevated or decreased in hypoparathyroidism, primary hypothyroidism, Graves disease, secondary hypothyroidism, and primary hyperthyroidism?
Definition

HyPOparathyroidism    ↓PTH, ↓ Ca, ↑Phosphorous (from med book)

Primary HyPOthyroidism    ↑ TSH due to abnormal thyroid gland


Grave’s Disease    ↑ thyroid hormone due to AI stimulation


Secondary HyPOthyroidism    ↓ to normal TSH due to pituitary dysfxn, iatrogenic or AI disease (Hashimoto’s thyroiditis)


Primary HyPERthryoidism    ↑ thyroid hormone due to thyroid tumor

 

 

  TSH is released from pituitary gland to stimulate thyroid gland 

Term
Which endocrine disorder(s) is (are) of autoimmune etiology?
  
Definition
 Graves disease (a type of hyperthyroidism)
      Hashimoto’s thyroiditis (a type of secondary hypothyroidism)
     Addison’s Disease (Hypoadrenocorticism; other etiologies also possible)
Term
. What causes secondary hyperparathyroidism?
   
Definition
Kidney disease →↓Active Vit D → ↓ GI Ca absorption → ↓ Serum Ca → ↑ PTH
Term
. What causes Addison’s disease?
  
Definition
 loss of corticosteroid
Term
What are the various conditions often associated with diabetes mellitus?
  
Definition
 Diabetic sialadenosis (salivary gland enlargement), oral candidiasis, delayed healing, rapidly progressing perio disease, gingival
    enlargement & erythema, zygomycosis (fungal infections), benign migratory glossitis, xerostomia, sailadenosis
Term
. What causes the premature tooth loss in hypophosphatasia?  
 
Definition
  Lack of cementum on root’s surface
Term
What are the clinical and histologic findings in pyostomatitis vegetans
   
Definition
Clinical: multiple yellow pustules (may have snail track appearance), swollen & red oral mucosa, papillary
    folds & grooves w/ pebbly surface, most common on facial gingiva, vestibule, buccolabial mucosa, not
    uncomfortable
    Histologic: eosinophilia
Term
. What is the cranial nerve mechanism involved with glossopharyngeal neuralgia?
  
Definition
Ninth; usually unknown etiology; may be due to neoplasms at skull base or aneurysms in the posterior
    cranial fossa.
Term
. What cranial nerve is involved with Bell’s palsy?
Definition
    Seventh
Term
What is helpful in the diagnosis of trigeminal neuralgia?
   
Definition
anatomic trigger point; extreme, paroxymal pain; carbamazepine- used to treat
Term
What are the trigger points of trigeminal neuralgia and glossopharyngeal neuralgia and which neuralgia is more likely to have intraoral trigger points?
Definition

    Trigeminal neuralgia -  nasolabial fold, lip vermilion, periorbital skin; intraoral - intraoral rare but alveolar ridge has been seen
    Glossopharyngeal neuralgia - internal trigger points (except ear canal) so more likely to have intraoral
        trigger points

 

Pain precipitated by:
Talking
Chewing
Swallowing
Yawning
Touching the tonsillar area on the affected side

Term
What are the characteristics of a cluster headache?
   
Definition
severe, lacinating painful esp around eyes, occurs at same time of day; lasts 15 min -3 hrs up to 8x day unilateral (simulates toothache); pt. may pace restlessly, no trigger zone, alcohol or nitroglycerin may     precipitate pain, occurs over period of weeks then months of remission
Term
What kind of test(s) help(s) provide a positive diagnosis for myasthenia gravis?
   
Definition
↑ serum AChR  (acetylcholine receptor ) antibody levels
improved strength after edrophonium injection (cholinesterase inhibitor)
Ice pack test: neuromuscular transmission improves w/ cold (test eye)
Term
What techniques/medicines help relieve the symptoms of burning mouth syndrome?
  
Definition
Anti-anxiety meds (e.g., clonazepam)
    Fight fire w/ fire → tobasco sauce or capsaicin cream
     Correct underlying condition
    Supportive techniques if idiopathic type
Term
What are the clinical signs and symptoms of Frey syndrome (auriculotemporal syndrome)?
Definition
  Unilateral sweating, flushing, brought on by chewing food & salivating
    Parasymp fibers are cut during surgery (e.g., parotid) and incorrectly regenerate along sympathetic
    pathways
Term
What is the mechanism and initial pathologic site of osteoarthritis?
  
Definition
 Slower and less complete replacement of chondroblasts and chondrocytes in joint cartilage leaving bone
    exposed; may have inflammatory components; occurs in weight bearing joints (hip)
Term
What kind of test(s) help(s) provide a positive diagnosis of rheumatoid arthritis?
 
Definition
  ↑Rheumatoid factor (RF); only 50% have + ANA titer
Term
What is the mechanism and initial pathologic site of rheumatoid arthritis?
   
Definition
Elevated titer of antinuclear antibodies
    Initially a synovitis (attack against synovial membrane)
    Pannus (destruction of cartilage and bone by collagenases released by reactive macrophage-laden
        fibroblasts
Small joints in hands and feet are initial sites of involvement
Term
What are the characteristic findings in the joints of osteoarthritis and rheumatoid arthritis?
Definition
OA - thinned cartilage and bone ends rub together; “joint mice” form
    RA - bone erosion and swollen/inflamed; “rice bodies” form