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oral path 2 final > Immune-Mediated Mucocutaneous 3 & 4 > Flashcards

Immune-Mediated Mucocutaneous 3 & 4 Flashcards

1
Q

T/F: Cutaneous Lichen Planus is a CHRONIC disorder

A

true

  • typically disease course is 1-2 years
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2
Q

what groups are most likely to develop Cutaneous Lichen Planus?

A

Affects adults (1%), 30-60 years of age

Female predilection

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3
Q

what are the clinical characteristics of cutaneous lichen planus?

A

A) Purple pruritic polygonal papules

B) Wickham’s striae

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4
Q

where are Cutaneous Lichen Planus lesions typically found?

how long does this disease typically last?

A

Flexor surface of wrists, lumbar region, shins

Typically, 1-2 yr disease course

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5
Q

T/F: Oral Lichen Planus only occurs in association with Cutaneous Lichen Planus

A

False

May occur alone or with skin lesions

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6
Q

what groups are most likely to develop Oral Lichen Planus?

A

Adults, with female predilection (3:2)

Same as cutaneous

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7
Q

where in the mouth is Oral Lichen Planus found?

which form of oral lichen planus is more COMMON?

which form is more SYMPTOMATIC?

A

Bilateral buccal mucosa, tongue, gingiva

Reticular form is most common

Erosive form is most symptomatic

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8
Q

Which condition has a longer disease course: oral, or cutaneous, Lichen Planus

A

ORAL Lichen planus lasts longer

  • Protracted disease course, years-decades
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9
Q

what do the lesions from RETICULAR oral lichen planus look like?

A

Reticular LP: interlacing white lines

white color due to hyperkeratosis

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10
Q

where in the mouth would Reticular Oral Lichen Planus show patchy keratosis and atrophy?

A

Dorsal tongue

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11
Q

what are the clinical manifestations of EROSIVE Oral Lichen planus?

A

shallow ulcers, peripheral erythema and radiating white lines

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12
Q

T/F: C. albicans infections may be superimposed on BOTH Reticular and Erosive forms of oral lichen planus

A

true

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13
Q

what are the main histopathological finding for Oral Lichen Planus?

A

1) Hyperkeratosis
2) Alternating atrophy/thickening of spinous layer

3) pointed rete ridges
4) degeneration of the basal cell layer
5) band-like infiltrate of lymphocytes

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14
Q

How is Oral Lichen Planus treated?

give treatment for BOTH reticular and erosive forms

A

A) Management includes treatment of any associated candidiasis

B) Reticular LP: usually requires no therapy

C) Erosive LP: should be treated with one of the stronger topical corticosteroids; systemic steroids usually not needed

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15
Q

what is the prognosis for oral lichen planus?

Is OLP precancerous?

A

A) Prognosis is good

  • Controversy exists regarding the OLP as premalignant condition
  • Current best molecular evidence does not support a premalignant potential
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16
Q

Oral lichen planus can be confused with what other broad disease category?

A

Lichenoid Mucositis

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17
Q

what are some types of Lichenoid Mucositis?

A

A) Lichenoid drug reaction
B) lichenoid amalgam reaction
C) oral mucosal cinnamon reaction

D) lichenoid foreign body gingivitis
E) oral lesions of GVHD or lupus erythematous

F) some epithelial dysplasias

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18
Q

Which disease is “The most common of the significant immune-mediated systemic diseases”

A

Systemic Lupus Erythematosus

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19
Q

How many Americans are affected by Systemic Lupus Erythematosus?

What population groups are at highest risk?

A

Approximately 1.5 million affected in U.S.

Women affected 8 to 10 times more often than men
Average age at diagnosis – 31 years

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20
Q

what are the INITIAL symptoms of Systemic Lupus Erythematosus?

A

Protean (variable) manifestations initially:

1) Fever, weight loss, arthritis, fatigue, malaise
2) Malar “butterfly” rash
3) Skin lesions flare with sun exposure

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21
Q

what are the effects of Systemic Lupus Erythematosus on the organ systems?

A

1) Renal involvement (40-50%), renal failure
2) Cardiac involvement is common, particularly pericarditis
3) 50% have vegetations on heart valves (Libman-Sacks endocarditis)

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22
Q

What is often the most significant clinical complication for Systemic Lupus Erythematosus (SLE)?

A

renal failure

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23
Q

Oral lesions are present in _______% of SLE

A

5-25%

24
Q

what are the characteristics of SLE oral lesions?

A
  • May appear as non-specific or lichenoid ulcerations/keratotic areas
  • Affect palate, buccal mucosa, or gingiva
25
Q

T/F: the treatment for SLE oral lesions is usually topical corticosteroids

A

true

  • secondary candidiasis infection is common
26
Q

what are the microscopic characteristics of Systemic Lupus Erythematosus (SLE)?

A

A) Lichenoid pattern microscopically

B) Positive lupus band test – deposition of immunoreactants at BMZ of normal skin

C) Anti-nuclear antibodies present (95%), especially to dsDNA

27
Q

how is SLE treated?

A

Treatment includes:
A) decreasing patient’s exposure to UV light
B) Mild disease- NSAID agents or anti-malarial drugs
C) more significant cases- Systemic corticosteroids

28
Q

what conditions would warrant the use of Systemic corticosteroids to treat SLE?

A
  • arthritis
  • pericarditis
  • nephritis
  • thrombocytopenia
29
Q

what is the prognosis for SLE?

what about the 5 and 10-year survival rates?

A

Prognosis is variable

5-year survival is 95%
20-year survival falls to 75%

30
Q

Do men or women have a worse prognosis for SLE?

A

Worse for MEN

more common in women, hits men harder

31
Q

_________________ is related to SLE, but almost exclusively affects skin and mucosa

A

Chronic Cutaneous Lupus

32
Q

what is the clinical presentation for Chronic Cutaneous Lupus?

A

Scaly, erythematous patches appear on sun-exposed skin of the head and neck

Scarring and atrophy as these lesions heal

33
Q

what do the ORAL lesions of CCL resemble?

A

Oral lesions resemble erosive Lichen Planus

34
Q

T/F: The clinical appearance of CCL skin lesions usually is characteristic

A

true

35
Q

what are the microscopic features of CCL lesions?

A

Microscopic examination of oral lesion shows lichenoid mucositis and vasculitis

36
Q

Which form of lupus typically presents with anti-nuclear antibodies? which does not?

A

SLE = anti-nuclear antibodies

CCL = typically none

37
Q

How is CCL treated?

A

A) Treatment usually includes avoiding excessive UV light exposure

B) Topical corticosteroids or calcineurin inhibitors are often helpful with either cutaneous or oral lesions

C) More difficult cases may require systemic anti-malarial drug therapy or low-dose thalidomide

38
Q

T/F: the prognosis for Chronic Cutaneous Lupus is much better than for Systemic lupus

A

true

39
Q

Approximately ___% of CCLE patients may transform to SLE

A

5

40
Q

what % of Chronic Cutaneous Lesions will resolve after several years?

A

50%

41
Q

________________ is also known as “Scleroderma”

A

Systemic Sclerosis

42
Q

what causes Systemic Sclerosis?

A

inappropriate deposition of dense collagen

43
Q

what is the prevalence of Systemic Sclerosis?

A

10-20 cases/million

44
Q

what groups are at highest risk for developing systemic sclerosis?

A

Women affected 3-5 times more frequently than men

adult age group

**same as all the others in this section

45
Q

what are the clinical characteristic features of Systemic Sclerosis?

(NOT the oral characteristics)

A

A) Raynaud’s phenomenon; claw-like deformity of fingers; acro-osteolysis

B) Diffuse smooth, hard texture of skin

46
Q

what organ systems can be effected by Systemic Sclerosis?

A

Pulmonary, renal, cardiac and gastrointestinal FIBROSIS may be seen

— Pulmonary fibrosis leads to hypertension and heart failure

47
Q

what are the ORAL characteristics of Systemic Sclerosis?

A

1) Microstomia, with pinched, “purse-string” appearance of mouth
2) Dysphagia with esophageal involvement
3) Diffuse widening of periodontal ligament
4) Resorption of posterior ramus, condyle, coronoid process or chin in 10-20%

48
Q

what antibodies are present during Systemic Sclerosis?

A

A) Serologic studies show autoantibodies directed against Scl-70 (topoisomerase 1)

B) Anticentromere antibodies

49
Q

Anticentromere antibodies are associated more with ______ syndrome than with Systemic Sclerosis

A

associated more with CREST syndrome

50
Q

what are some possible treatment options for Systemic Sclerosis?

A
  • No uniformly effective treatment -

Supportive care includes:
esophageal dilation, calcium channel blockers to ease Raynaud’s symptoms, ACE inhibitors to control hypertension

51
Q

what is the prognosis for both limited & diffuse Systemic Sclerosis?

A

10 year survival:

  • 75-80% with limited disease
  • 55-60% with diffuse disease
52
Q

_____________ is a mild variant of Systemic Sclerosis

A

CREST Syndrome

53
Q

what does “CREST” stand for?

A 
Calcinosis cutis
Raynaud’s phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasia
54
Q

what population group is most likely to develop CREST syndrome?

A

Women

6th to 7th decade of life

55
Q

how is CREST syndrome treated?

how does the prognosis compare to Systemic Sclerosis?

A

A) Symptomatic care, similar to systemic sclerosis

B) Better prognosis than systemic sclerosis

56
Q

what causes Libman-Sacks endocarditis?

A

Systemic Lupus erythematous

oral path 2 final (3 decks)

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