Immune-mediated Mucocutaneous Disease 2 Flashcards

1
Q

Why are steroids used to treat auto-immune disorders/conditions?

A

steroids suppress the immune response

-auto immune conditions result from an over-active immune response

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2
Q

What percentage of Erythema Multiforme results from an unknown cause/etiology? Preceding infection? Medication related?

A

50% unknown
25% infection
25% meds

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3
Q

What are the two types of preceding infections that are common with Erythema Multiforme?

A

viral (herpes)

bacterial (mycoplasma pneumoniae)

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4
Q

What type of medications are more likely to initiate Erythema Multiforme?

A

antibiotics

analgesics

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5
Q

What is the difference between EM Minor and EM Major?

A

EM Minor: skin or mucosa only (one site)

EM Major: at least two mucosal sites + skin involvement

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6
Q

__________ Syndrome is an EM condition that involves two mucosal sites and ~10% of skin involvement.

A

Stevens-Johnson

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7
Q

What is Lyell’s Disease?

A

variation of Erythema Multiforme with diffuse bullous involvement of skin and mucosa (~30% skin involvement)
aka- Toxic Epidermal Necrolysis

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8
Q

Of the two major EM disorders:

Stevens-Johnson Syndrome involves ____% of skin; Lyell’s disease involves _____% of skin.

A

SJ 10%

LD 30%

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9
Q

What are the clinical features of Erythema Multiforme?

A
  • hemorrhagic crusting of lips **
  • widespread oral ulcers with ragged margins
  • target lesions of skin***
  • bright red conjunctiva
  • sloughing of skin (feet especially)
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10
Q

Which oral sites are typically spared in Erythema Multiforme?

A

gingiva

hard palate

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11
Q

What is a “target” lesion?

A
  • concentric-looking macules with crusted central ulceration
  • typical of Erythema Multiforme
  • affects extremities first
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12
Q

True or False: There is no useful immunologic pattern for diagnosing Erythema Multiforme

A

True

  • light microscopic features are typical but not diagnostic
  • keratinocyte destruction
  • mixed inflammatory infiltrate
  • perivascular inflammation
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13
Q

How is DIF used in diagnosing Erythema Multiforme?

A

DIF is used to rule-out other conditions but is not diagnostic

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14
Q

What is the treatment of choice for mild cases of EM?

A

-supportive care (analgesics, soft diet, hydration, steroid syrup)

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15
Q

What is the treatment of choice for minor EM?

A
  • controversial

- corticosteroids given empirically

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16
Q

What is the treatment of choice for major EM? Compare Stevens-Johnson vs. Toxic Epidermal Necrolysis.

A

SJS: if medication related, discontinue the drug
TEN: managed in burn unit, IV human immunoglobulin shows promise. Avoid corticosteroids = detrimental

17
Q

True or False: Corticosteroids are used to treat Toxic Epidermal Necrolysis.

A

False, some studies show them to be detrimental

18
Q

True or False: Cases in which EM recurs are usually reported in the Autumn or Spring.

A

True

19
Q

What percentage of EM recurs?

A

20%

20
Q

What is the treatment of choice for recurrent EM?

A
  • identify the initiating factor (HSV or drug)

- if HSV initiated, continuous antiviral therapy

21
Q

What is the prognosis for EM?

A

mild to moderate = good
major = 2-10% mortality rate
TEN = 34% mortality rate

22
Q

Which has a better prognosis, SJS or TEN?

A

SJS prognosis > TEN prognosis

23
Q

How common is erythema migrans?

A

1-3% of the population

24
Q

True or False: Erythema migrans is probably immune mediated.

A

True

25
Q

True or False: Erythema Migrans is often seen with fissured tongue.

A

True, 20-30% of cases

26
Q

How often does erythema migrans occur with fissured tongue?

A

20-30%

27
Q

Benign Migratory Glossitis typically ______ and ______.

A

waxes and wanes

28
Q

Where is erythema migrans characteristically found?

A

dorsal and lateral anterior 2/3 of the tongue

29
Q

True or False: Benign Migratory Glossitis does not develop on surfaces outside the tongue.

A

False, may develop on other non-keratinized mucosal surfaces.
“ectopic geographic tongue”

30
Q

Describe the appearance of Geographic tongue (benign migratory glossitis/erythema migrans).

A

multiple, well-demarcated zones of erythema surrounded by a slightly elevated yellow-white serpentine borders
(swelling causes parakeratin to be lifted in specific areas and sheared off)

31
Q

What is the histopathologic finding associated with erythema migrans?

A

“psoriasiform mucositis”

parakeratosis with extensive microabscess formation in the superficial spinous layer

32
Q

What is the treatment of choice for Erythema Migrans?

A
  • no treatment is generally necessary
  • some patients complain of sensitivity to hot or spicy foods when lesions are active (use of stronger topical corticosteroid gel)