final Flashcards
what is due to defect in normal keratinization of oral mucosa?
white sponge nevus
white sponge nevus affects what and what are the signs and symptoms
WSN affects buccal mucosa mainly but can also affect nasal, esophageal, laryngeal, anogenital mucosa
asymptomatic thick white appearance
Biopsy of WSN shows what?
WSN: parakeratosis w acanthosis, perinuclear eosinophilic condensation which is pathognomic to WSN
WSN tx and prognosis
WSN tx is NOT necessary, harmless condition Good prognosis
what % of Peutz Jeghers syndrome pts are new mutations?
what gene is affected in Peutz Jeghers
35% new mutations!
SKT 11 gene affected which encodes for a serine threonine kinase
what is the frequency of Peutz Jeghers syndrome cases
1:100,00- 1:200,000 births
Peutz Jeghers syndrome dead giveaway sign?
Are the polyps from PJ pre-cancerous?
PJ= freckle like lesions which develop on hands, periorifical skin, and oral mucosa
Polyps in GI tract are NOT precancerous like in Gardners Syndrome
Peutz Jeghers syndrome pts have how many times INC risk for cancer?
what happens to their bowels?
PJ pts 18X more at risk for cancer!
Their bowel obstruct due to intussusception (telescoping of proximal segment)
What is the frequency of hereditary hemorrhagic telangiectasia?
lesions of HHT are most common where?
1: 10,000
lesions of HHT most common on vermillion zone of lips, tongue, and buccal mucosa
what is the initial clue to HHT?
where else may HHT be seen besides orally?
frequent spontaneous epistaxis
HHT may also be seen on hands and feet, GI mucosa, genitourinary mucosa, and conjunctival mucosa
what % of HHT pts have AV fistulas of lungs?
of liver?
of brain?
HHT AV fistula in lungs= 30%!
AV fistulas in liver = 30%
AV fistulas in brain 10-20%
3 out of what 4 things must be present for diagnosis of HHT
recurrent, spontaneous epistaxis
telangiectasias of mucosa and skin
AV malformation involving lung, liver, or brain
family history of HHT
What is the tx for mild HHT?
moderate HHT?
Severe HHT?
Mild HHT: no Tx
moderate HHT: selective cryotherapy or electrocautery of bothersome lesions
Severe: septal dermoplasty
HHT brain AV fistulas that get bacteremia from dental procedures occur in what % of pts? so what should be done for these pts?
HHT AV fistulas of brain bacteremia occurs 1% of pts : so these pts should get prophylactic ABs
What is the mortality rate for HHT pts?
what is the mortality rate for HHT pts with brain abscesses?
HHT mortality rate= 1-2% due to blood loss
HHT mortality rate w brain abscess= 10%
Pemphigus vulgaris autoantibodies destroy what? so a split occurs where in the tissue?
PV= autoAB destroy desmosomes
PV inhibits adherence so a split develops within the epithelium
PV occurs in who?
what percent of PV pts present w oral lesions? what are the oral lesions?
Rare no sex predilection but in older pts around 50.
More than 50% have oral lesions of superficial ragged erosions and ulcerations
where does PV occur?
PV is positive for what?
PV occurs on any oral mucosa, flacid bull on skin, intact oral blisters rare
+ Nikolsky sign!
in PV what is the 1st to show and last to go? common oral lesion?
ORAL LESIONS!
shallow ulcerations right along the marginal gingival area
DIF is used for what and what animal?
IFF is used for what and what animal?
DIF: detects autoABs bound to pts tissues DIF= goat IIF: detects autoAbs circulating in blood IIF: monkey
For each disease is DIF +/-? IIF +/-?
PV?
MM pemphigoid?
Bullous pemphigoid?
PV: + DIF and + IIF
MM pemphigoid: + DIF, - IIF
Bullous pemphigoid: + DIF and + IIF
PV shows what microscopically?
PV= INTRAepithelial clefting in basal layer and acantholysis- breakdown of spinous layer
Mortality rate of PV?
tx of PV?
PV= 5-10% mortality rate!
PV tx= systemic corticosteroids
what is 2x more common than PV and has a 2:1 female predilection? what is the average age?
Mucous membran pemphigoid: 2:1 females
average age 50-60 y/o
In MM pemphigoid what tissue is affected?
Is oral scarring common?
MM pemphigoid affects any mucosal surface and skin
cutaneous lesion and conjunctival scarring is common
ORAL Scarring is RARE!