Final Flashcards
Group of inherited disorders in which TWO or more ectodermally derived structures do not develop normally or fail to develop is:
-Examples of ectoderm structures:
Ectodermal Dysplasia
-Skin, hair, nails, teeth, sweat glands
Hypohidrotic Ectodermal Dysplasia causes ______ due to _______
Heat intolerance
..reduced sweat glands
What causes fine, sparse blonde hair and oligodontia (less than 6 teeth)
Hypohidrotic Ectodermal Dysplasia
When is White Sponge Nevus usually noticed?
- It is due to:
- Appearance
At birth or early childhood
- Defect in the normal keratinization of the oral mucosa
- Asymptomatic, thick, white appearance of the buccal mucosa bilaterally
How do you diagnose White Sponge Nevus?
-Tx:
Exfoliative Cytology (better than biopsy) -No tx necessary
Peutz-Jeghers Syndrome..
- __% represent new mutations
- ____ gene is affected
- Frequency
- tx
- 35%
- SKT11
- 1 in 100,000-200,000
- genetic counseling
What is characterized by FRECKLE-line lesions on hands, periorifacial skin (mouth, nose, anus, genitals) AND causes GI POLYPS
Peutz-Jeghers Syndrome
In Peutz-Jeghers Syndrome (unline Gardner Syndrome), the polyps are NOT ____, but these patients have __x chance of cancer than general population
precancerous
-18x
Hereditary Hemorrhagic Telangiectasia
- What is a telangiectasia?
- frequency:
- Due to mutation in a gene that plays a role in:
- Initial clue to diagnoses
- Prognosis
- small collection of dilated capillaries
- 1 in 10,000
- blood vessel wall integrity
- frequent epistaxis (nose bleed)
- Good, but 1-2% mortality dues to blood loss (or brain abscess)
Pt has a 1-2mm red papule on vermillion border of lip that blanch with diascopy, this is:
Hereditary Hemorrhagic Telangiectasia
What can cause ArterioVenous problems
HHT (Hereditary Hemorrhagic Telangiectasia)
In Pemphigus Vulgaris, ______ destroy ____
autoantibodies…desmosomes
Pemphigus
- age
- sex
- prognosis
- 4th-6th decade
- none
- fatal if not treated
Pemphigus
- __% present with oral lesions
- Appears as:
- 50%
- Ragged erosions and ulcerations
What is the Nikolsky Sign?
-Which disease has it
- Skin rubs off easily
- Pemphigus
In pemphigus, oral lesions are “______ and _____”
“First to show and last to go”
In pempigus, direct AND indirect immunoflouresnce (IF) studies will be:
positive
Pemphigus tx:
-prognosis
SYSTEMIC corticosteriods (NOT topical) often with azathioprene -5-10% mortality
Mucous Membrane Pemphigoid is aka:
Cicatrical (“scarring”) pemphigoid
Which is 2-4x more common, pemphigus or pemphigoid
pemphigoid more common
Pemphigoid..
- age:
- sex:
- older (50-60)
- 2:1 female
Pemphigoid may affect:
- Scarring usually seen with:
- Often presents as:
- May see _______ intraorally
any mucosal surface
- conjuctival (symblepharon) lesions
- desquamative gingivitis
- intact blisters
With pemphigoid ____ scarring is rare. The most significant aspect of the disease is ____ involvement
oral
-ocular
Pemphigoid..
- What do you submit for biopsy
- In what solutions?
- DIF is usually __, IIF is usually __
- Normal mucosa, 0.5-1cm away from ulceration
- Michel’s and formalin
- DIF + , IIF -
Pemphigoid tx…
- if oral lesions only:
- if ocular involvement
- fatal?
- oral: topical steroids, tetracycline or dapsone
- ocular: immunosuppressive therapy
- rarely
Bullous pemphigoid..
- Usually affects what age?
- ____ lesions are seen primarily, rarely ____
- Initial complaint is usually _____
- Old (75-80)
- cutaneous, rarely oral
- pruritus (itching)
Erythema Multiforme is an ____, self-limiting ulcerative disorder
ACUTE
Erythema Multiforme predilection
Young adult female
Erythema Multiforme can be caused by
HSV infection
Erythema Multiforme minor:
-Major
minor: skin or oral mucosa
- major: at least two mucosal sites plus skin involvement
What disease causes Stevens-Johnson and Toxic Epidermal Necrolysis
Erythema Multiforme
What causes “target” lesions of skin
Erythema Multiforme
Erythema Multiforme…
- prognosis for minor:
- major:
- Toxic Epidermal Necrolysis (TEN):
- minor: good
- major: 1-5% mortality
- TEN: 25-30% mortality
What disease is recurrent in 20% of cases in autumn and spring?
Erythema Mutiforme
Erythema Migrans (benign migratory glossitis) is aka:
- Occurs in ___ of population (relatively common)
- Tx
“geographic tongue”
- 1-3%
- No tx
Pts usually report waxing and waning lesions often seen with fissured tongue (so they might complain from spicy foods)
Erythema Migrans (benign migratory glossitis)
Cutaneous Lichen Planus is a chronic _______ disorder
immune-mediated
Cutaneous Lichen Planus age range:
- Sex
- __yr disease course
30-60
- female predilection
- 1-2
What causes purple pruritic polygonal papules with WICKHAM’S STRIAE
Cutaneous Lichen Planus
Cutaneous Lichen Planus usually affects what parts of body?
flexor surfaces of wrists, lumbar region, shins
Oral Lichen Planus sex predilection?
-Female
Oral Lichen Planus affects which parts of mouth
-Appearance:
Bilateral buccal mucosa, tongue, gingiva
-interlacing white lines
Oral Lichen Planus…
- most common form:
- most symptomatic form
- common: reticular
- symptomatic: erosive
What is the most common significant systemic disease?
- How many affected in US
- Sex?
- Average age
Lupus Erythematous
- 1.5 million
- Female 10x
- 31
What causes fever, weight loss, arthritis, fatigue, and malaise?
Lupus Erythematous
What causes malar “butterfly” rash
Lupus Erythematous
What is the most significant complication of Lupus Erythematous?
- This organ is involved in __% of patients
- What organ is also commonly involved?
- __% have vegeatations on these valves
Renal failure
- 50%
- Heart
- 50%
__-__% of Lupus Erythematous pts present oral lesions
5-25
Lupus Erythematous appears as:
-Area
lichenoid ulcerations/keratotic areas
-palate, buccal mucosa, gingiva
Lupus Erythematous tx:
Topical corticosteroids
Lupus Erythematous prognosis…
- 5 yr survival rate
- 20 yr survival rate
- Prognosis is worse for:
- 5 = 95%
- 20 = 75%
- men
Chronic Cutaneous Lupus appearance and area:
-Is prognosis better or worse than lupus erythematous
Scaly, erythematous patches on sun-exposed skin of head and neck
-much better
What is the relatively rare condition characterized by inappropriate deposition of dense collagen
Systemic Sclerosis (scleroderma)
Systemic Sclerosis..
- Prevelance (per year)
- Sex
- 10-20 per million annually
- Women 3-5x more
What causes Raynaud’s Phenomenom (deformity of fingers)
Systemic Sclerosis
What is the worst complication of Systemic Sclerosis
Causes fibrosis of major organs (pulmonary fibrosis leads to hypertension and heart failure)
What causes Microstomia “purse string” appearance of mouth, diffuse widening of PDL, and resorption of posterior mandible and condyle
Systemic Sclerosis
Systemic Sclerosis 10yr survival rate with diffuse disease
55-60%
CREST is the milder version of _____
-Sex and age
Systemic Sclerosis
-Women 6th-7th decade
What is CREST
Calcinosis cutis, Raynaud’s phenomenen, Esophageal dysfunction, Sclerodactyly, Telangiectasia
3 highest risk sites for oral cancer (SCC)
- Ventro-lateral tongue
- floor of mouth
- anterior tonsillar pillars/retromolar area
High risk features of oral cancer
larger than 1cm
-non-homogneous patches/plaques
What can’t Conventional Oral Exam (COE) do?
Can’t determine lesions that will progress to cancer
Does the ViziLite enhance visual detection of oral cancer beyond COE alone?
NO!
VELscope is is good at identifying:
- Good at high-risk/low-risk discrimination?
- Has a very high false _____ rate
the MARGINS of the lesion
- NO!
- false positive
Which device uses 3 different colors for exam?
Identafi
What color does Identafi use to show vasculature
Amber
Initial follow-up for suspicious oral lesions should be between __- __
7-21 days
