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AU 16- Oral Path Exam 4 > Immune-mediated Mucocutaneous Disease 3 > Flashcards

Immune-mediated Mucocutaneous Disease 3 Flashcards

1
Q

What are the two general forms of lichen planus?

A
  1. Cutaneous

2. Oral

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2
Q

What are the two forms of oral lichen planus?

A
  1. reticular

2. erosive

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3
Q

What is cutaneous lichen planus?

A

-chronic immune-mediated disorder that causes purple polygonal pruritic papules with Wickham’s Striae (lacey-looking)

CLP = PPPP

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4
Q

Cutaneous lichen planus typically appears on the flexor surfaces of _____, _____, and the _____ region.

A

wrists
shins
lumbar

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5
Q

Who is more likely affected by cutaneous lichen planus?

a. 15 year old male
b. 15 year old female
c. 45 year old female
d. 45 year old male

A

c. 45 year old female
(3: 2 female predilection, 30-60 years old average)

***older women LIKE (lichen) CUTE (cutaneous) things and PLANts (planus)

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6
Q

Which form of oral lichen planus will present with pain?

A

erosive

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7
Q

True or False: Oral lichen planus does not ever present with skin lesions.

A

False, it may or may not

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8
Q

Which form of oral lichen planus is most common?

A

reticular

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9
Q

Where does oral lichen planus present?

A

bilaterally

  • buccal mucosa
  • tongue
  • gingiva
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10
Q

What is the classic appearance of Lichen Planus?

A

Wickham’s Striae- lacy white patches

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11
Q

Which gingival presentation confuses the diagnosis of lichen planus due to it being a feature of several diseases?

A

Desquamative Gingivitis

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12
Q

Name conditions that presents with desquamative gingivitis. How do you differentiate between various diseases?

A 
Mucous Membrane Pemphigoid
Bullous Pemphigoid
Lichen Planus
Pemphigus Vulgaris
Lupus Erythematous

Immunofluorescence DIF/IFF

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13
Q

What are the microscopic finding of Oral Lichen Planus?

A 
hyperkeratosis
thickening of spinous layer
absent or pointed rete ridges
degeneration of basal cell layer
band-like infiltration of lymphocytes
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14
Q

True or False: Oral Lichen Planus is a “clinical” diagnosis.

A

True

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15
Q

How is Oral Lichen Planus managed?

A

Treat the candida if present
Reticular: No therapy, roughness but no pain
Erosive: topical corticosteroids

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16
Q

True or False: Prognosis of Oral Lichen Planus is fair due to malignant potential.

A

False,
no evidence to support malignant potential
prognosis is GOOD

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17
Q

Cutaneous lichen planus typically lasts _____ to _____ years. Oral Lichen Planus lasts ________.

A 
cutaneous = 7-10 years
oral = lifetime management, waxes and wanes
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18
Q

Name several conditions that mimic lichen planus.

A
  • lichenoid drug reaction
  • oral mucosal cinnamon reaction
  • lichenoid foreign body gingivitis
  • oral lesions of graft vs host
  • oral lesions of leukoedema
  • some epithelial dysplasias
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19
Q

Lichenoid conditions will show histopathologic _____ center formation.

A

germinal

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20
Q

What is the most common of the significant immune-mediated systemic diseases?

A

Systemic Lupus Erythematosus

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21
Q

_____ are affected by SLE 8-10 times more often.

A

Women (especially women of color)

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22
Q

What is the average age of diagnosis for systemic lupus erythematosus?

A

31 years

SLE….S1E…3 letters and a 1 = 31 years old

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23
Q

What is the initial presentation of Systemic Lupus Erythematosus? What is the later manifestation?

A

Initially: “Protean manifestations” = fever, weight loss, arthritis, fatigue, malaise
Later: Malar “butterfly” rash that spares the nasolabial folds

24
Q

What is a significant complication with Systemic Lupus Erythematosus? How often does this occur?

A 
Renal Involvement (may lead to kidney failure)
40-50% of cases

SLEK = SLE + Kidney Failure= F for Forty to Fifty

25
Q

Cardiac involvement is common with SLE, particularly ______.

A

periocarditis

26
Q

_____% of SLE patients have sterile _____ on heart valves. This is called _______.

A

50%
vegetations
Libman-Sacks Endocarditis

27
Q

True or False: Oral lesions are extremely common with SLE.

A

False, oral lesions occur in 5-25%

28
Q

Oral lesions associated with SLE will be non-specific or _____. Lesions will affect which aspects of the oral cavity?

A

lichenoid
palate, buccal mucosa, gingiva
(responds to topical corticosteroids)

29
Q

Diagnosis of Systemic Lupus Erythematosus is based on clinical, histopathologic, _____ and _____ findings.

A

immunopathologic

serologic

30
Q

True or False: A positive Lupus Band Test is diagnostic for Systemic Lupus Erythematosus.

A

False, not specific to SLE

deposition of immunoreactants at the basement membrane zone

31
Q

Serum studies show ______ present in ___% of SLE patients.

A

Anti-Nuclear Antibodies
95%

non-specific to SLE but used in screening

32
Q

What is the treatment for SLE?

A

decreasing patient’s exposure to UV light
non-steroid anti-inflammatory agents or anti-malarials
systemic corticosteroids (significant cases)

33
Q

True or False: The prognosis for SLE is worse for women.

A

false, worse for men

34
Q

What is the most common cause of death in SLE?

A

renal failure

35
Q

What is the 5-year survival rate? 15 year?

A

5 year = 95%

15 year = 75%

36
Q

True or False: Chronic Cutaneous Lupus is a different disease than SLE.

A

True

37
Q

Chronic Cutaneous Lupus almost exclusively affects the _____ and ____.

A

skin and mucosa

38
Q

Where does chronic cutaneous lupus appear? What do the lesions look like?

A

on sun-exposed skin of the head and neck

-scaly, erythematous patches with scarring and atrophy (cosmetic issues-picture Seal’s face)

39
Q

True or False: Chronic Cutaneous Lupus is typically diagnosed through a process of elimination.

A

True, shows lichenoid mucositis and vasculitis, serologic tests are negative for anti-nuclear antibodies, skin lesion appearance is characteristic

40
Q

What is the treatment for Chronic Cutaneous Lupus?

A
  • avoid excessive UV light exposure
  • topical corticosteroids
  • systemic anti-malarial drug therapy or low does thalidomide for more difficult cases
41
Q

True or False: Chronic Cutaneous Lupus has a poorer prognosis than SLE.

A

False, much better than SLE (due to SLE renal failure)

42
Q

Approximately ____% of Chronic Cutaneous Lupus patients may transform to SLE. Approximately ____% of cases will resolve after several years.

A

5%

50%

43
Q

____ ________ is a relatively rare condition that is characterized by inappropriate deposition of dense collagen.

A

Systemic Sclerosis

44
Q

True or False: Women are more likely to be affected by systemic sclerosis.

A

True (3 times more likely)

probably immunologically mediated

45
Q

What are the common characteristics of Systemic Sclerosis?

A
  1. Raynaud’s
  2. Claw-like deformation of fingers + ulceration of tips (“sclerodactyly”)
  3. Acro-osteolysis
  4. Diffuse smooth, hard texture of skin (hidebound/”scleroderma”)
  5. Pulmonary Hypertension due to pulmonary, Renal, cardiac, and GI fibrosis
  6. Pinched nose/face and microstomia
46
Q

What is acro-osteolysis?

A

destruction of digit tips, including bone

47
Q

What is a common cause of death associated with Systemic Sclerosis?

A

heart failure

48
Q

Which condition presents with a pinched “purse string” appearance of the mouth?

A

Systemic Sclerosis

49
Q

What is the radiographic presentation of systemic sclerosis?

A
  • diffuse/generalized widening of the PDL
  • resorption of posterior ramus/condyle/coronoid/chin (10-20%)
  • sometimes tooth resorption
50
Q

Serologic studies of Systemic Sclerosis show autoantibodies directed against _____. The _____- antibodies are more often associated with CREST Syndrome.

A

Scl-70 (topoisomerase I)

anticentromere

51
Q

True or False: There is no good treatment for Systemic Sclerosis.

A

True, supportive care (esophageal dilation, Ca-channel blockers for Raynaud’s, ACE inhib. for hypertension)

52
Q

_____% of patients with Systemic Sclerosis will survive 8 years after diagnosis.

A

30-50%

3+5 = 8 years

53
Q

What is CREST syndrome?

A

a milder variant of systemic sclerosis that has antibodies directed against anticentormeres
-affects women in 6th-7th decade

54
Q

What does CREST stand for?

A 
Calcinosis cutis (calcium deposits under skin)
Raynauds
Esophageal dysfunction
Sclerodactyly
Telangiectasia
55
Q

True or False: CREST has a better prognosis than Systemic Sclerosis.

A

True

56
Q

What is the survival rate for CREST syndrome?

A

80% 6-year survival

50% 12-year survival

AU 16- Oral Path Exam 4 (6 decks)

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