Immune-mediated Mucocutaneous Disease 2 Flashcards
Why are steroids used to treat auto-immune disorders/conditions?
steroids suppress the immune response
-auto immune conditions result from an over-active immune response
What percentage of Erythema Multiforme results from an unknown cause/etiology? Preceding infection? Medication related?
50% unknown
25% infection
25% meds
What are the two types of preceding infections that are common with Erythema Multiforme?
viral (herpes)
bacterial (mycoplasma pneumoniae)
What type of medications are more likely to initiate Erythema Multiforme?
antibiotics
analgesics
What is the difference between EM Minor and EM Major?
EM Minor: skin or mucosa only (one site)
EM Major: at least two mucosal sites + skin involvement
__________ Syndrome is an EM condition that involves two mucosal sites and ~10% of skin involvement.
Stevens-Johnson
What is Lyell’s Disease?
variation of Erythema Multiforme with diffuse bullous involvement of skin and mucosa (~30% skin involvement)
aka- Toxic Epidermal Necrolysis
Of the two major EM disorders:
Stevens-Johnson Syndrome involves ____% of skin; Lyell’s disease involves _____% of skin.
SJ 10%
LD 30%
What are the clinical features of Erythema Multiforme?
- hemorrhagic crusting of lips **
- widespread oral ulcers with ragged margins
- target lesions of skin***
- bright red conjunctiva
- sloughing of skin (feet especially)
Which oral sites are typically spared in Erythema Multiforme?
gingiva
hard palate
What is a “target” lesion?
- concentric-looking macules with crusted central ulceration
- typical of Erythema Multiforme
- affects extremities first
True or False: There is no useful immunologic pattern for diagnosing Erythema Multiforme
True
- light microscopic features are typical but not diagnostic
- keratinocyte destruction
- mixed inflammatory infiltrate
- perivascular inflammation
How is DIF used in diagnosing Erythema Multiforme?
DIF is used to rule-out other conditions but is not diagnostic
What is the treatment of choice for mild cases of EM?
-supportive care (analgesics, soft diet, hydration, steroid syrup)
What is the treatment of choice for minor EM?
- controversial
- corticosteroids given empirically
What is the treatment of choice for major EM? Compare Stevens-Johnson vs. Toxic Epidermal Necrolysis.
SJS: if medication related, discontinue the drug
TEN: managed in burn unit, IV human immunoglobulin shows promise. Avoid corticosteroids = detrimental
True or False: Corticosteroids are used to treat Toxic Epidermal Necrolysis.
False, some studies show them to be detrimental
True or False: Cases in which EM recurs are usually reported in the Autumn or Spring.
True
What percentage of EM recurs?
20%
What is the treatment of choice for recurrent EM?
- identify the initiating factor (HSV or drug)
- if HSV initiated, continuous antiviral therapy
What is the prognosis for EM?
mild to moderate = good
major = 2-10% mortality rate
TEN = 34% mortality rate
Which has a better prognosis, SJS or TEN?
SJS prognosis > TEN prognosis
How common is erythema migrans?
1-3% of the population
True or False: Erythema migrans is probably immune mediated.
True
True or False: Erythema Migrans is often seen with fissured tongue.
True, 20-30% of cases
How often does erythema migrans occur with fissured tongue?
20-30%
Benign Migratory Glossitis typically ______ and ______.
waxes and wanes
Where is erythema migrans characteristically found?
dorsal and lateral anterior 2/3 of the tongue
True or False: Benign Migratory Glossitis does not develop on surfaces outside the tongue.
False, may develop on other non-keratinized mucosal surfaces.
“ectopic geographic tongue”
Describe the appearance of Geographic tongue (benign migratory glossitis/erythema migrans).
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)
What is the histopathologic finding associated with erythema migrans?
“psoriasiform mucositis”
parakeratosis with extensive microabscess formation in the superficial spinous layer
What is the treatment of choice for Erythema Migrans?
- 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)
