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Summer 2017-Oral Medicine Final > Mucosal, Oral, and Cutaneous Disease > Flashcards

Mucosal, Oral, and Cutaneous Disease Flashcards

1
Q

Acne Vulgaris

A
  1. very common
  2. hormones
  3. medications
  4. skin bacteria
  5. stress
  6. genetic susceptibility
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2
Q

Open comodones

A
  1. black-heads

2. open to the air (oxidized)

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3
Q

Closed comodones

A

white-heads

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4
Q

Nodule-cystic acne

A
  1. leads to scarring

2. inflammatory

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5
Q

Topical Acne Tx

A
  1. comedolytics (target the comedones)

2. anti-inflammatories (benzoyl peroxide, clindamycin, dapsone)

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6
Q

Systemic Tx for Acne

A
  1. oral antibiotics (tetracycline, doxycycline, minocycline)
  2. combined oral contraceptives
  3. isotretinoin
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7
Q

Non-melanoma skin cancers

A
  1. includes basal cell carcinomas (BCC) and squamous cell carcinomas (SCC)
  2. most COMMON malignancy in humans
    * *BCCs: 80%
    * *SCCs: 20%
  3. 3.5 million cases annually in the US, affects ALL races
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8
Q

Risk factors for non-melanoma skin cancer

A
  1. fair skin (skin type I or II)
  2. age
  3. male
  4. sun or UV exposure
    * *SCC: cumulative
    * *BCC: intermittent
    * *melanoma: tanning beds
  5. ionizing radiation
  6. genetic syndromes
  7. immunosuprresion
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9
Q

SCC (specific) skin cancer risk factors

A
  1. arsenic
  2. HPV
  3. PUVA
  4. smoking
  5. chronic wounds
  6. long-standing skin disease
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10
Q

Basal Cell Carcinoma

A
  1. most common type
  2. most often on head and neck
  3. pick, pearly papule with a rolled edge
  4. chronic course, slow growth with ulceration
  5. rarely metastasizes
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11
Q

Nodular Basal Cell Carcinoma

A
  1. classic type

2. 50-80% of all types

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12
Q

Pigmented Basal Cell Carcinoma

A
  1. non-painful, don’t bleed
  2. can be confused with melanoma
  3. must biopsy
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13
Q

Superficial BCC Skin Cancer

A
  1. second most common
  2. 15% of BCCs
  3. favor the trunk and extremities
  4. located in top layers of skin
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14
Q

Invasive/Morpheaform BCC skin cancer

A
  1. locally aggressive sub-type
  2. may appear scar-like
  3. often a histologic determination
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15
Q

Actinic Keratoses

A
  1. pre-malignant or pre-cancerous
  2. 10% develop into SCC over 10 years (if untreated)
    * *60% of SCCs develop from AKs
  3. same risk factors as SCC
  4. prevention: sunscreen/low fat diet
  5. tx: destruction/topical immunotherapy
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16
Q

Squamous Cell Carcinoma Skin Cancer

A
  1. 300,000 per year in US

2. most common skin cancer in AA

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17
Q

Risk factors for SCC skin cancer

A
  1. cumulative long term exposure to UV light
  2. radiation
  3. immunosuppression
  4. chronic ulceration, scar, HPV, carcinogens
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18
Q

Keratoacanthoma

A
  1. rapid growth
  2. clinically tame
  3. variant of SCC
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19
Q

SCC in situ

A
  1. Bowen’s disease
  2. elderly
  3. sun-exposed skin
  4. de novo or from AKs
  5. head and neck&raquo_space;extremities
  6. tender to the touch, not a lot of depth
"BEST"
B: Bowen's Disease
E: elderly
S: sun
T: tender to touch
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20
Q

High Risk Sites for Skin Cancer

A
  1. trunk and extremities: 2 cm and greater

2. cheeks, forehead, neck, and scalp: 1 cm and greater

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21
Q

Indications for Mohs Surgery

A
  1. high risk sites
  2. recurrent
  3. poorly delineated or incompletely removed
  4. pt on immunosuppression
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22
Q

Melanoma

A
  1. incidence in whites has tripled in last thirty years

2. lifetime risk in 1 in 70

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23
Q

Nodular Melanoma

A
  1. 15-20% of melanomas
  2. no radial growth phase–>rapid growth
  3. trunk and legs most common
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24
Q

Acral-Lentiginous Melanoma

A
  1. melanoma on the toes or fingers

2. most common type in AA, Asians, Hispanics

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25
Q

Exzema herpeticum

A
  1. all paules looks the same
  2. punched out erosion
  3. associated with herpes
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26
Q

Topical Steroids

A
  1. main tx with atopic derm
  2. body locations: risk of side effects directly related to steroid strength, inversely related to skin thickness
    * *thinnest skin: eyelids, scrotum
    * *thin skin: face, axillae, groin
    * *average skin: trunk, arms, legs
    * *thicker skin: hands, feet
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27
Q

Allergic contact dermatitis is a __ (type of hypersensitivity reaction)

A

delayed type hypersensitivity reaction to previously sensitized chemical

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28
Q

Irritant contact dermatitis

A

local toxic effect from an irritant chemical

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29
Q

Poison Ivy

A
  1. very itchy, difficult to control

2. vesicles (skin breaks apart with fluid)

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30
Q

Nickel

A
  1. costume jewelry, pants rivets

2. can develop a rash all over body if severe enough

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31
Q

Cradle Cap

A

seborrheic dermatitis

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32
Q

__ is the most common malignancy in humans

A

Basal Cell Carcinoma

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33
Q

__ (percent) of SCCs develop from AKs

A

60%

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34
Q

AKs risk factors are the same as __

A

SCC

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35
Q

__ may be prevented with sunscreen/low fat diet

A

AKs

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36
Q

Tx for AKs is __

A

destruction or topical immunotherapy

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37
Q

AKs are also known as __

A
  1. solar keratoses
  2. senile keratoses
  3. “pre cancers”
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38
Q

Where do AKs occur?

A

sun damaged skin

  1. scalp
  2. face
  3. top of ears
  4. hands
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39
Q

What is the appearance of AKs?

A
  1. rough
  2. gritty pink to red
  3. macule/papule with angular border
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40
Q

__ (percent) of AKs turn into invasive SCC

A

0.1%

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41
Q

__ is the second most common form of skin cancer

A

squamous cell carcinoma

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42
Q

SCC is most often found __

A

head, neck, dorsal hands

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43
Q

SCC appears __

A
  1. pink to red scaly/ulcerated
  2. enlarging papule or nodule
  3. may be tender
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44
Q

Metastases of SCC is most common when it involves __

A
  1. high risk sites (lip, ear)
  2. immunosuppression
  3. lesions arising in scars, sites of inflammation
  4. size > 2 cm
  5. injured/chronically diseased skin
  6. perineural invasion
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45
Q

Erythroplasia of Queyrat is SCC on the __

A

penis

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46
Q

Tx for Skin Cancer

A
  1. topical
    * *retinoids
    * *5-fluorouracil (Efudex)
    * *imiquimod (Aldara)
  2. photodynamic therapy
    * *blue light treatment
  3. cryotherapy
  4. surgical excision
    * *margins depend on type
    * *Moh’s
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47
Q

__ is the median age of diagnosis for melanoma

A

53 years

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48
Q

__ is the most common cancer in women aged 25-29

A

melanoma

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49
Q

Risk factors for melanoma

A
  1. family history of MM
  2. person history of MM
  3. atypical nevi
  4. numbers of banal nevi
  5. history of excessive UV and sunburns
  6. fair skin
  7. immuno-suppression
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50
Q

Lentigo Maligna-Melanoma

A
  1. form of in situ melanoma (confined to epidermis, but full potential to invade)
  2. uncommon large malignant pigmented patch on chronically sun exposed skin
  3. usually long radial growth phase
  4. need large (broad) shave biopsy to diagnose, excellent margin control to remove
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51
Q

Lentigo Maligna-Melanoma makes up __ (percent) of melanomas

A

4-15%

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52
Q

Tx for Melanoma

A
  1. wide excision
  2. Moh’s surgery
  3. sentinel lymph node biopsy
  4. staging workup
  5. Adjuvant therapy
    * *Interferon-A
    * *vaccines
    * *isolated limb perfusion of chemotherapy
  6. CLOSE FOLLOW UP every three months
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53
Q

Atopic Dermatitis

A
  1. common inflammatory condition of the skin
  2. AKA “eczema”
  3. associated with seasonal allergies, asthma, food allergies
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54
Q

Eczema

A
  1. means “to break out, boil over”
  2. group of dermatoses with characteristic histologic/clinical features
  3. not a diagnoses in itself
  4. broad term–>important to distinguish “kind of eczema”
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55
Q

Prevalence of Atopic Dermatitis

A
  1. 0.6-20% of individuals

2. higher in children: 95% develop before age 15, but 40-60% outgrow it

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56
Q

Inheritance of Atopic Dermatitis

A
  1. one parent with atopy=50% chance
  2. two parents with atopy=79% chance
  3. polygenic
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57
Q

How does atopic dermatitis affect quality of life?

A
  1. decreased self-esteem
  2. poor sleep/work performance
  3. depression
  4. isolation
  5. poor scoring on QOL
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58
Q

Atopic Dermatitis generally starts as __ (age)

A

infant or young child

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59
Q

Atopic Dermatitis is located on the __ of infants

A

face, extensor surfaces

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60
Q

Atopic Dermatitis is located on the __ of young kids

A

LESS on face, more on extensor surfaces

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61
Q

Atopic Dermatitis is located on the __ of older kids and adults

A

flexural surfaces

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62
Q

Atopic Dermatitis - Clinical Features

A
  1. xerosis
  2. very pruritic
  3. excoriations
  4. lichenification
  5. colonized with MSSA/MRSA
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63
Q

Adults with Atopic Dermatitis tend to __, while children __

A
  1. adults: run a chronic course

2. children: improve with time

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64
Q

Complications with Atopic Dermatitis include __

A
  1. bacterial super-infection
  2. eczema herpeticum
  3. erythroderma
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65
Q

The two main side effects of topical steroids are __

A
  1. skin atrophy (reversible if caught early)

2. absorption (excessive local and systemic)

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66
Q

What complications are associated with topical steroids?

A
  1. atrophy (thinning)
  2. bruising
  3. stretch marks
  4. steroid acne
  5. localized infections
  6. perioral dermatitis
  7. contact dermatitis
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67
Q

__ is related to sebum-production and commensal yeast Malassezia

A

seborrheic dermatitis

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68
Q

Seborrheic Dermatitis - Clinical Features

A
  1. scalp
  2. face (forehead, eyebrowns, upper eyelids, nasolabial and melolabial folds)
  3. neck and central chest
  4. retro-auricular creases, under breasts, in pannus folds, groin
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69
Q

Seborrheic Dermatitis appears as __

A
  1. sharply demarcated greasy, pink-yellow to red-brown patches/plaques
  2. bran-like scale
  3. chronic, relapsing course
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70
Q

Severe cases of seborrheic dermatitis are associated with __

A

HIV or Parkinson’s disease

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71
Q

Tx for Seborrheic Dermatitis

A
  1. anti-yeast tx

2. anti-inflammatory tx when more red and inflamed

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72
Q

Psoriasis

A
  1. chronic inflammatory disorder of the skin

2. affects children and adults

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73
Q

The subtypes of psoriasis include __

A
  1. plaque-type
  2. guttate
  3. psoriatic arthritis
  4. pustular psoriasis
  5. erythroderma
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74
Q

What can trigger psoriasis?

A
  1. infection (streptoccus)
  2. emotional/physical stress
  3. trauma
  4. certain medications (Beta blockers, hydroxychloroquine, ibuprofen)
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75
Q

What is associated with psoriasis?

A
  1. obesity
  2. metabolic syndrome
    * *high BP
    * *excess body fat
    * *high blood pressure
  3. increased risk for cardiac mobility and mortality in those who have psoriasis
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76
Q

Psoriasis - Topical Tx Options

A
  1. steroids
  2. calcineurin inhibitors
  3. Vitamin D analogues
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77
Q

Psoriasis - Systemic Tx Options

A
  1. narrowband UVB phototherapy

2. methotrexate, cyclosporine, acitretin

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78
Q

Perioral Dermatitis

A
  1. inflammatory condition on face
  2. usually made worse with fluorinated topical steroids or fluoride toothpaste
  3. DOESN’T involve vermilion border
  4. can involve perinasal and periocular skin
  5. pruritic
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79
Q

Tx for Perioral Dermatitis

A
  1. remove offending agents
  2. gentle, fragrance-free skin care
  3. topical antiboties - metronidazole, erythromycin
  4. oral antibiotics - doxycycline, minocyline (adults), and erythromycin
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80
Q

Lichen Planus

A

inflammatory disease of skin, hair, nails, mucous membranes

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81
Q

The “P’s” of Lichen Planus

A
  1. planar polygonal pruritic pink

2. purple papules or plaques

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82
Q

Lichen Planus is commonly found on the __

A
  1. legs
  2. ankles
  3. wrists
  4. genitalia
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83
Q

Lichen Planus is associated with __

A
  1. viruses (Hep C)
  2. drugs
    * *Beta-Blockers
    * *ACE inhibitors
    * *Thiazide diuretics
    * *Anti-malarials
    * *gold and metals
    * *Penicillamine
  3. Hep B vaccine
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84
Q

__ (percent) of LP patients have nail involvement

A

10%

  1. thinning of nail plates
  2. longitudinal ridging
  3. pterygium formation (scarring)

isolated nail LP may also occur

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85
Q

Mucosal Lichen Planus is found __

A
  1. oral (most common)
  2. genital
  3. pharynx, esophagus, GI tract
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86
Q

Two types of Mucosal Lichen Planus are __

A
  1. reticulated

2. erosive

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87
Q

Reticulated Mucosal Lichen Planus

A
  1. linear, lace-like pattern of tiny white papules
  2. buccal mucosa (most common)
  3. typically asymptomatic
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88
Q

Erosive Mucosal Lichen Planus

A
  1. gingiva or tongue

2. typically painful

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89
Q

Tx - Mucosal Lichen Planus

A
  1. topical steroids
  2. topical calcineurin inhibitors
  3. NBUVB phototherapy
  4. systemic
    * *oral corticosteroids
    * *metronidazole
    * *anti-malarials
90
Q

Pemphigus Vulgaris

A
  1. acquired
  2. autoimmune bulbous disease
  3. auto-antibodies to desmosomal proteins (desmoglein 1 and 3)
  4. intraepidermal blistering
91
Q

Flaccid/easily ruptured bullae, oral and mucosal lesions, and a positive Nikolsky’s sign is indicative of __

A

Pemphigus Vulgaris

Stevens-Johnson Syndrome also has a positive Nikolsy sign

92
Q

Tx for Pemphigus Vulgaris

A
  1. prednisone
  2. asathiorprine
  3. Mycophenolate mofetil
  4. Rituximab
93
Q

Mucous Membrane Pemphigoid

A
  1. autoimmune blistering condition
  2. mostly affects older people
  3. recurrent blistering of mucous membranes, also skin
  4. pts develop scars, strictures, synechiae, blindness
  5. proteins affected
94
Q

MMP - Diagnosis

A
  1. biopsies: H and E, direct immunofluorescence
  2. indirect immunofluroescence
    * *evaluate blood for circulating antibodies
  3. age-appropriate cancer-screening
95
Q

MMP - Tx

A
  1. prednisone

2. other immunosuppresant systemic agents

96
Q

__ is the most common autoimmune bullous dermatosis

A

Bullous Pemphigoid (BP)

97
Q

Bullous Pemphigoid occurs primarily in the __ (age)

98
Q

BP usually starts with __ (disease description)

A

highly pruritic urticaria without blistering

99
Q

BP usually develops into __ (disease description)

A

tense blisters with serous or hemorrhagic fluid

100
Q

The blisters of BP can be described as __

A

firm and stable

101
Q

BP - Diagnosis

A
  1. H and E and direct immunofluorescence

2. IIF usually shows linear staining of epidermal side of salt-split skin

102
Q

BP - Tx

A
  1. mild cases: high potency steroids

2. severe disease: oral steroids, other immunosuppresants

103
Q

__ and __ are the affected proteins involved in BP

A
  1. BP-180

2 BP-230

104
Q

Pemphigus vulgaris/Bullous pemphigus has a better prognosis

A

Bullous pemphigus

105
Q

Dermatitis Herpetiformis is a consequence of __

A

gluten sensitivity

106
Q

Dermatitis Herpetiformis is much less/more prevalent than celiac disease

A

much less common

107
Q

What bodily reactions can be caused by gluten in grains?

A
  1. immune reaction in small bowel (alters the mucosa)
  2. leads to antibody formation
  3. antibodies can enter blood, attach to skin
  4. dermatitis herpetiformis
108
Q

Dermatitis herpetiformis is pruritic/non-pruritic

A

VERY pruritic

109
Q

Dermatitis Herpetiformis is commonly found on __ (body parts)

A
  1. favors extensor surfaces: forearms and thighs
  2. butt
  3. papules, vesicles, numerous erosions
110
Q

The best tx for Dermatitis Herpetiformis is __

A

complete avoidance of gluten

111
Q

Dapsone is an effective tx for relief of DH (almost immediately for itching), but it doesn’t affect __

A

gut or production of autoantibodies

112
Q

Dermatitis Herpetiformis - Work Up

A
  1. biopsy for H and E
  2. direct immunofluorescence
  3. H and E shows numerous neutrophils/PMNs in upper dermis
  4. DIF shows deposits of IgA in papillary dermis
  5. serum transglutaminase 3 (TTG) typically elevated
113
Q

Linear IgA disease

A
  1. acquired autoimmune bullous disease
  2. chronic bullous disease of childhood
  3. IgA antibodies act against components of basement membrane (BP 180 and BP230)
114
Q

__ are triggers for linear IgA disease

A
  1. infection

2. medications (vancomycin)

115
Q

Linear IgA Disease - Clinical Features

A
  1. urticarial plaques on the skin that form blisters
    * *clustered at edges of lesions
    * *may be pruritic
  2. oral lesions
116
Q

Linear IgA Disease - Work-Up

A
  1. biopsy

2. immunofluorescence

117
Q

Linear IgA Disease - Tx

A
  1. dapsone
  2. corticosteroids
  3. tetracylines
118
Q

Erythema Multiforme (EM)

A
  1. typically self-limiting, acute disease of skin and mucosa
    * *can be recurrent
  2. hypersensitivity/immune reaction
119
Q

Abrupt development of targetoid lesions (3 distinct zones/rings) is characteristic of __

120
Q

EM favors the __ (locations on the body)

A
  1. upper body
  2. extremities and face
  3. palms of hands
121
Q

EM mucosal lesions are typically found on __

A

lips/oral mucosa >ocular > genital

122
Q

Severe cases of EM may be accompanied by __

A
  1. fevers

2. arthralgias

123
Q

EM - Tx

A
  1. If HSV related: treat with acyclovir or valacyclovir
  2. topical steroids
  3. systemic immunosuppressives
    * *prednisone
    * *methylprednisone
    * *azathioprine
    * *dapsone
    * *cyclosporine
124
Q

Stevens-Johnson Syndrome (SJS)

A

rare, potentially fatal, acute reaction

  1. adults: meds implicated in majority of cases
  2. children: illnesses and meds
125
Q

Stevens-Johnson Syndrome is characterized by __

A

mucosal and skin lesions due to extensive keratinocyte death

126
Q

The average mortality rate of Stevens-Johnson Syndrome is __

127
Q

__ is a risk factor of Stevens-Johnson Syndrome

A

immunosuppression (HIV, lymphoma)

128
Q

Stevens-Johnson Syndrome - Prodrome Clinical Features

A

1 to 3 days before cutaneous signs

  1. fever
  2. URI symptoms
  3. painful skin
  4. dysphagia
129
Q

Stevens-Johnson Syndrome - Skin Lesions

A
  1. pink macules–>dusky red or purpuric–>gray–>vesicles/bulla–>irregular erosions
  2. appear on trunk, spreads to arms/neck/face
  3. positive Nikolsky sign
  4. may take hours to a few days
  5. <10% BSA
130
Q

Stevens-Johnson Syndrome - Mucosal Lesions

A

present in 90%

  1. painful erosions that coalesce
  2. lips, mouth, throat, esophagus
  3. nose
  4. eyes
  5. genitalia
131
Q

Toxic Epidermal Necrolysis

A
  1. exists on spectrum with SJS
    * *>30% BSA
  2. mortality rate approaches 25-35% of patients
    * *typically due to systemic infection
132
Q

The risk of developing Toxic Epidermal Necrolysis is 1000-fold higher if you have __ (disease)

133
Q

SJS/TEN Tx

A
  1. stop offending tx
  2. stymptomatic cares
    * *eye care
    * *skin protection
    * *electrolyte and fluid monitoring
  3. immunosuppression
134
Q

Behcet’s Disease

A
  1. rare, multi-system inflammatory condition
  2. can affect any individual
  3. especially common for people on the “Silk Road”
  4. genetic and evironmental fators
  5. difficult to diagnose
135
Q

Behcet’s Disease Triad

A
  1. oral ulcers
  2. genital ulcers
  3. uveitis
136
Q

__ (disease) is associated with the Silk Road

A

Behcet’s Disease

137
Q

HLA-B51 allele (80% of Asians) is associated with __

A

Behcet’s Disease

138
Q

Behcet’s Disease - Clinical Features

A

Recurrent oral ulceration (painful aphthous-like ulcers, testing negative for herpes) in almost 100% of patients

PLUS

Two of the Four:

  1. recurrent genital ulcerations
  2. eye inflammation
  3. skin lesions
  4. positive pathery test
139
Q

Behcet’s disease - Tx

A
  1. Colchicine and dapsone
  2. systemic steroids
  3. thalidomide
  4. methotrexate
  5. azathiprine
  6. cyclosporine
140
Q

Sweet Syndrome

A

acute febrile neutrophile dermatitis

141
Q

Sweet Syndrome - Features

A
  1. fever, malaise
  2. edematous
  3. pink papules, plaques, blisters
  4. may involve oral mucosa and eyes
142
Q

Sweet Syndrome is associated with __

A
  1. infection
  2. malignancies
  3. inflammatory bowel disease
  4. autoimmune disease
  5. certain meds
  6. pregnancy
143
Q

Sweet Syndrome - Tx

A
  1. treat underlying cause
    * *malignancy
    * *infection
    * *inflammatory bowel disease
  2. prednisone
    * *improves rapidly
144
Q

Ectodermal Dysplasia

A
  1. collection of inerited disorders affecting
    * *hair
    * *teeth
    * *nails
    * *sweat glands
  2. depends on mutation
    * *can be X linked, AD, or AR
145
Q

Basal Cell Nevus Syndrome

A
  1. AKA Gorlin Syndrome
  2. AD inheritance
  3. affects PTCH gene
  4. 1 in 50,000 to 150,000 affected
146
Q

Gorlin Syndrome - Findings

A
  1. BCC’s of skin
  2. odontogenic keratocytes (seen at young age)
  3. rib and vertebrae anomalities
  4. calcification of falx cerebri
  5. skeletal abnormalities: bifid ribs, kryphoscoliosis
  6. frontal and temproparietal bossing
147
Q

Nevoid BCC’s are often mistakenly diagnosed as __

A

melanocytic nevi (many are brown)

148
Q

Nevoid BCC’s can look like __

149
Q

Nevoid BBC’s tend to develop around __ (time of life)

A

puberty

“Think that Neville was in puberty when he was on Harry Potter”

150
Q

Gorlin Syndrome - Tx

A

mostly symptomatic

  1. enucleation of odontogenic keratocysts
  2. removal of BCC’s
  3. vismodegib
    * *help decrease size and need for surgical tx
    * *everybody has side effects
151
Q

Gardner Syndrome is AKA

A

AKA familial polyposis

152
Q

Gardner Syndrome - Clinical Features

A
  1. osteomas (favor mandible and maxilla)
  2. supernumerary teeth, multiple caries
  3. fibromas of skin
  4. congenital hypertrophy of retinal pigment epithelium
  5. colorectal cancer by age 40 in almost every patient
153
Q

Neurofibromatosis (NF)

A
  1. autosomal dominant disorder

2. two types: NF1 or NF2

154
Q

NF1

A
  1. mutation in neurofibromin (RAS activation)

2. 1 in 3500 births (50% are new mutations)

155
Q

NF2

A
  1. mutation in Merlin or Schwannomin
    * *RAS activation via over-activation of serine/threonine kinase
  2. 1 in 40,000 births (66% have affected parent)
156
Q

Cafe-au-lait macules, freckling of axillary/inguinal areas, neurobifromas, plexiform neurofibromas, lisch nodules in eye, optic nerve gliomas, and skeletal dysplasia are characteristic of __

A

NF1 (must have two or more)

157
Q

Crowe’s Sign are cafe-au lait macules in the groin and maxilla and characteristic of __

158
Q

Learning disability, ADD, macrocephaly, brainstem gliomas, meningiomas are characteristic of __

159
Q

There is a __ (percent) chance of developing malignancy due to NF1

160
Q

Tuberous Sclerosis Complex

A
  1. AD with variable expressivity
  2. affects 1 in 6000 people
  3. defects in TSC1 (hamartin) and TSC2 (tuberin)
    * *suppress cell growth through regulation of several signaling pathways
    * *mTOR
161
Q

Tuberous Sclerosis is characterized by development of hamartomas in __ (tissues)

A
  1. skin
  2. brain
  3. eye
  4. heart
  5. kidneys
  6. lungs
  7. bones
162
Q

Cutaneous Manifestations of Tuberous Sclerosis

A
  1. hypopigmented macules >3
  2. confetti skin lesions
  3. angiofibromas
  4. fibrous forehead plaque
  5. shagreen patch (CT nevus)
  6. non-traumatic ungual or peringual fibromas
  7. gingival fibroma
163
Q

Sturge-Weber Syndrome

A
  1. neuroectodermal syndrome

2. etiology is unclear

164
Q

Sturge-Weber Syndrome is characterized by __

A
  1. capillary malformation (port-wine stain), distribution of first branch of trigeminal nerve (V1)
  2. soft-tissue overgrowth can be seen
165
Q

Sturge-Weber Syndrome is associated with __ (diseases)

A
  1. leptomeningeal angiomatosis

2. glaucoma

166
Q

Sturge-Weber Syndrome - CNS Findings

A
  1. seizures are most common feature seen in first year of life
  2. early onset, seizure intensity associated with future cognitive delay
  3. headaches
  4. behavioral problmes
  5. stroke-like episodes
167
Q

SWS - Lentomeningeal Angiomatosis

A
  1. ipsilateral from vascular stain
  2. cerebral atrophy
  3. enlargment of choroid plexus and venous abnormalities
  4. MRI is modality of choice (CT can pick up changes too)
168
Q

“Tram Track” calcification in the cerebral cortex is associated with __

A

Leptomeningeal Angiomatosis (in Sturge-Weber Syndrome)

can be seen in early infancy

169
Q

Ocular Changes - Sturge Weber Syndrome

A
  1. 60% of patients
  2. glaucoma is most common
  3. vascular malformations on/around eye
  4. nevus of ota
  5. blindness
170
Q

Pseudoxanthoma Elasticum

A
  1. AKA Gronblad-Strandberg syndrome
  2. AR (ABCC6)
  3. prevalence–>25,000 to 100,000
171
Q

Calcification of elastic fibers is characteristic of __

A

Pseudoxanthoma elasticum

  1. mild to deep dermis
  2. media and intima of mid-sized arteries
  3. Bruch’s membrane of eye
172
Q

Thin yellowish papules and plaques are characteristic of __

A

pseudoxanthoma elasticum

  1. flexural areas (neck, antecubital/popliteal fossae, wrists, groins)
  2. tends to develop over first and second decade
173
Q

Plucked chicken skin is associated with __

A

pseudoxanthoma elasticum

174
Q

Angioid streaks are present in __

A

pseudoxanthoma elasticum

  1. typically present in first decade
  2. virtaully all patients by age 30
  3. normally asymptomatic
  4. increased neovascularization–>prone to hemorrhage–>scarring–>progressive loss of vision
  5. vision changes by age 40 (leads to legal blindness)
175
Q

Cardiovascular changes - pseudoxanthoma elasticum

A
  1. progressive calcification of arteries
    * *atheromatous plaques
    * *intermitten claudication
    * *renovascular hypertension
    * *angina
    * *MI
    * *stroke
  2. GI bleeding as result of gastric/intestinal vessels
  3. mitral valve prolapse
176
Q

Monitoring PXE

A
  1. skin: can’t do much
  2. eyes: biannual fundoscopic exam, no smoking/head trauma/heavy straining
  3. CV: baseline EKG and ECHO, healthy lifestyle, annual cardiac exam
177
Q

Gorlin syndrome is characterized by calcifications of the __ (body part)

A

Flax cerebri

178
Q

Bifid ribs and kryphoscolisosis is characteristic of__ (disease)

A

Gorlin syndrome

179
Q

Frontal and temproparietal bossing is characteristic of __ (disease)

A

Gorlin syndrome

180
Q

Rib and vertebrae anomalities are characteristic of __ (disease)

A

Gorlin syndrome

181
Q

Enculeation of odontogenic keratocysts is a tx for __(disease)

A

Gorlin syndrome

182
Q

Congential hypertrophy of retinal pigment epithelium is characteristic of __ (disease)

A

Gardner syndrome

183
Q

In __ (disease), cororectal cancer by age 40 is seen in almost every patient

A

Gardner syndrome

184
Q

Gorlin syndrome is mostly asymptomatic/symptomatic

A

symptomatic

185
Q

__ (type of cancer) is the most common malignancy in humans

186
Q

SCC/BCC is due to cumulative sun exposure

187
Q

SCC/BCC is due to intermittent sun exposure

188
Q

Arsenic

189
Q

HPV

190
Q

PUVA

191
Q

BCC is most often found on the __ (body part)

A

head and neck

192
Q

chronic course, slow growth and ulceration

193
Q

Superficial BCC favors __ (body part)

A

trunk and extremities

194
Q

Pre-malignant or pre-cancerous

A

Actinic Keratoses

195
Q

10% develop into SCC over 10 years (if untreated)

A

Actinic Keratoses

196
Q

same risk factors as SCC

A

Actinic Keratoses

197
Q

Prevention via sunscreen/low fat diet

A

Actinic Keratoses

198
Q

Tx via destruction/topical immunotherapy

A

Actinic Keratoses

199
Q

Form of in situ melanoma

A

Lentigo-Maligna Melanoma

200
Q

Uncommon large malignant pigmented patch on chronically exposed skin

A

Lentigo-Maligna Melanoma

201
Q

Long radial growth phase

A

Lentigo-Maligna Melanoma

202
Q

Inflammatory condition of face

A

perioral dermatitis

203
Q

Made worse with fluorinated topical steroids or fluoride toothpaste

A

perioral dermatitis

204
Q

Doesn’t involve vermillion border

A

perioral dermatitis

205
Q

Can involve perinasal and periocular skin

A

perioral dermatitis

206
Q

IBD/autoimmune disease/pregnancy

A

Sweet Syndrome

207
Q

Gorlin Syndrome

A

Basal Cell Nevus Syndrome

208
Q

AD inheritance

A

Basal Cell Nevus Syndrome

209
Q

PTCH syndrome

A

Basal Cell Nevus Syndrome

210
Q

Osteomas

A

Gardner Syndrome

211
Q

Supernumerary teeth, multiple caries

A

Gardner Syndrome

212
Q

Fibromas on skin

A

Gardner Syndrome

213
Q

Congenital hypertrophy of retinal pigment epithelium

A

Gardner Syndrome

214
Q

Colorectal cancer by age 40 in almost every patient Gardner Syndrome

A

Gardner Syndrome

215
Q

Hypopigmented macules >3

A

Tuberous Sclerosis

216
Q

Confetti skin lesions

A

Tuberous Sclerosis

217
Q

Angiofibromas

A

Tuberous Sclerosis

218
Q

Fibrous forehead plaque

A

Tuberous Sclerosis

219
Q

Shagreen patch (CT nevus)

A

Tuberous Sclerosis

220
Q

Non-traumatic ungual or peringual fibromas

A

Tuberous Sclerosis

221
Q

Gingival fibroma

A

Tuberous Sclerosis

Summer 2017-Oral Medicine Final (17 decks)

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