Immune-mediated Mucocutaneous Disease 2

Question 1

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

Answer 1

steroids suppress the immune response

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

Question 2

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

Answer 2

50% unknown
25% infection
25% meds

Question 3

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

Answer 3

viral (herpes)

bacterial (mycoplasma pneumoniae)

Question 4

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

Answer 4

antibiotics

analgesics

Question 5

What is the difference between EM Minor and EM Major?

Answer 5

EM Minor: skin or mucosa only (one site)

EM Major: at least two mucosal sites + skin involvement

Question 6

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

Answer 6

Stevens-Johnson

Question 7

What is Lyell’s Disease?

Answer 7

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

Question 8

Of the two major EM disorders:

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

Answer 8

SJ 10%

LD 30%

Question 9

What are the clinical features of Erythema Multiforme?

Answer 9

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

Question 10

Which oral sites are typically spared in Erythema Multiforme?

Answer 10

gingiva

hard palate

Question 11

What is a “target” lesion?

Answer 11

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

Question 12

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

Answer 12

True

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

Question 13

How is DIF used in diagnosing Erythema Multiforme?

Answer 13

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

Question 14

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

Answer 14

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

Question 15

What is the treatment of choice for minor EM?

Answer 15

• controversial

- corticosteroids given empirically

Question 16

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

Answer 16

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

Question 17

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

Answer 17

False, some studies show them to be detrimental

Question 18

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

Answer 18

True

Question 19

What percentage of EM recurs?

Answer 19

20%

Question 20

What is the treatment of choice for recurrent EM?

Answer 20

• identify the initiating factor (HSV or drug)

- if HSV initiated, continuous antiviral therapy

Question 21

What is the prognosis for EM?

Answer 21

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

Question 22

Which has a better prognosis, SJS or TEN?

Answer 22

SJS prognosis > TEN prognosis

Question 23

How common is erythema migrans?

Answer 23

1-3% of the population

Question 24

True or False: Erythema migrans is probably immune mediated.

Answer 24

True

Question 25

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

Answer 25

True, 20-30% of cases

Question 26

How often does erythema migrans occur with fissured tongue?

Answer 26

20-30%

Question 27

Benign Migratory Glossitis typically ______ and ______.

Answer 27

waxes and wanes

Question 28

Where is erythema migrans characteristically found?

Answer 28

dorsal and lateral anterior 2/3 of the tongue

Question 29

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

Answer 29

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

Question 30

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

Answer 30

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)

Question 31

What is the histopathologic finding associated with erythema migrans?

Answer 31

"psoriasiform mucositis" parakeratosis with extensive microabscess formation in the superficial spinous layer

Question 32

What is the treatment of choice for Erythema Migrans?

Answer 32

- 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)