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
Cardiac involvement is common with SLE, particularly ______.
periocarditis
26
_____% of SLE patients have sterile _____ on heart valves. This is called _______.
50% vegetations Libman-Sacks Endocarditis
27
True or False: Oral lesions are extremely common with SLE.
False, oral lesions occur in 5-25%
28
Oral lesions associated with SLE will be non-specific or _____. Lesions will affect which aspects of the oral cavity?
lichenoid palate, buccal mucosa, gingiva (responds to topical corticosteroids)
29
Diagnosis of Systemic Lupus Erythematosus is based on clinical, histopathologic, _____ and _____ findings.
immunopathologic | serologic
30
True or False: A positive Lupus Band Test is diagnostic for Systemic Lupus Erythematosus.
False, not specific to SLE | deposition of immunoreactants at the basement membrane zone
31
Serum studies show ______ present in ___% of SLE patients.
Anti-Nuclear Antibodies 95% **non-specific to SLE but used in screening**
32
What is the treatment for SLE?
decreasing patient's exposure to UV light non-steroid anti-inflammatory agents or anti-malarials systemic corticosteroids (significant cases)
33
True or False: The prognosis for SLE is worse for women.
false, worse for men
34
What is the most common cause of death in SLE?
renal failure
35
What is the 5-year survival rate? 15 year?
5 year = 95% | 15 year = 75%
36
True or False: Chronic Cutaneous Lupus is a different disease than SLE.
True
37
Chronic Cutaneous Lupus almost exclusively affects the _____ and ____.
skin and mucosa
38
Where does chronic cutaneous lupus appear? What do the lesions look like?
on sun-exposed skin of the head and neck | -scaly, erythematous patches with scarring and atrophy (cosmetic issues-picture Seal's face)
39
True or False: Chronic Cutaneous Lupus is typically diagnosed through a process of elimination.
True, shows lichenoid mucositis and vasculitis, serologic tests are negative for anti-nuclear antibodies, skin lesion appearance is characteristic
40
What is the treatment for Chronic Cutaneous Lupus?
- avoid excessive UV light exposure - topical corticosteroids - systemic anti-malarial drug therapy or low does thalidomide for more difficult cases
41
True or False: Chronic Cutaneous Lupus has a poorer prognosis than SLE.
False, much better than SLE (due to SLE renal failure)
42
Approximately ____% of Chronic Cutaneous Lupus patients may transform to SLE. Approximately ____% of cases will resolve after several years.
5% | 50%
43
____ ________ is a relatively rare condition that is characterized by inappropriate deposition of dense collagen.
Systemic Sclerosis
44
True or False: Women are more likely to be affected by systemic sclerosis.
True (3 times more likely) | *probably immunologically mediated*
45
What are the common characteristics of Systemic Sclerosis?
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
What is acro-osteolysis?
destruction of digit tips, including bone
47
What is a common cause of death associated with Systemic Sclerosis?
heart failure
48
Which condition presents with a pinched "purse string" appearance of the mouth?
Systemic Sclerosis
49
What is the radiographic presentation of systemic sclerosis?
- diffuse/generalized widening of the PDL - resorption of posterior ramus/condyle/coronoid/chin (10-20%) - sometimes tooth resorption
50
Serologic studies of Systemic Sclerosis show autoantibodies directed against _____. The _____- antibodies are more often associated with CREST Syndrome.
Scl-70 (topoisomerase I) | anticentromere
51
True or False: There is no good treatment for Systemic Sclerosis.
True, supportive care (esophageal dilation, Ca-channel blockers for Raynaud's, ACE inhib. for hypertension)
52
_____% of patients with Systemic Sclerosis will survive 8 years after diagnosis.
30-50% | 3+5 = 8 years
53
What is CREST syndrome?
a milder variant of systemic sclerosis that has antibodies directed against anticentormeres -affects women in 6th-7th decade
54
What does CREST stand for?
``` Calcinosis cutis (calcium deposits under skin) Raynauds Esophageal dysfunction Sclerodactyly Telangiectasia ```
55
True or False: CREST has a better prognosis than Systemic Sclerosis.
True
56
What is the survival rate for CREST syndrome?
80% 6-year survival | 50% 12-year survival