Developmental and Immune-mediated Mucocutaneous Conditions - part II Flashcards
mucous membrane pemphigoid (MMP) is also known as what?
cicatricial (scarring) pemphigoid
why does mucous membrane pemphigoid (MMP) resemble pemphigus vulgaris (PV)?
due to blister formation (i.e. pemphig”oid”)
T/F: pemphigus vulgaris (PV) is twice as common as mucous membrane pemphigoid (MMP)
false, MMP is twice as common as PV
what is the average age of pts dx’d with mucous membrane pemphigoid (MMP)?
50-60 y.o.
older age than PV
what is the gender predilection of mucous membrane pemphigoid (MMP)?
2:1 female predilection
where does mucous membrane pemphigoid (MMP) affect?
any mucosal surface, occasionally affects skin
scarring of mucous membrane pemphigoid (MMP) may appear where?
- skin
2. symblepheron (conjunctiva)
scarring on the oral mucosa due to mucous membrane pemphigoid (MMP) is common
false, rare
what might be seen in pts with mucous membrane pemphigoid (MMP) because the split is subepithelial?
intact intraoral blisters
clinical features of mucous membrane pemphigoid (MMP)
desquamative gingivitis
desquamative gingivitis
descriptive term: erythema, desquamation, ulceration
oral mucosal ulcers in pts with mucous membrane pemphigoid (MMP) are deeper than in pts with pemphigus vulgaris (PV)
true
what is the most significant aspect of mucous membrane pemphigoid (MMP)?
ocular involvement of sympblepheron
scarring of symblepheron in pts with mucous membrane pemphigoid (MMP) does what?
obstructs the orifices of glands that produces tears resulting in a dry eye
dry eye in pts with mucous membrane pemphigoid (MMP) leads to what?
keratinization of the corneal epithelium leading to blindness
entropion
upper eyelid turned inward
trichiasis
eyelashes rubbing against the eye
eyes of pts with mucous membrane pemphigoid (MMP)
- entropion
- trichiasis
- lower fornix is obliterated
biopsy of mucous membrane pemphigoid (MMP) must include what?
- generous sample of normal mucosa, 0.5-1 cm away from areas of ulceration/erythema, as epithelium easily strips off
- tissue should be submitted in both formalin and Michel’s solution
histo of mucous membrane pemphigoid (MMP)
SUBepithelial cleft formation
subepithelial cleft formation
separation of epithelium from CT at the basement membrane zone (BMZ)
immuno features of mucous membrane pemphigoid (MMP)
- linear deposition of immunoreactants at the BMZ
2. positive DIF; negative IIF
what percent of pts with mucous membrane pemphigoid (MMP) will have circulating autoantibodies?
only 5-25%
tx of mucous membrane pemphigoid (MMP) depends on what?
extent of involvement
tx of mucous membrane pemphigoid (MMP) oral lesions
- topic steroids - tetracycline/niacinamide or dapsone may be sufficient
- frequent dental prophylaxis, every 3-4 months
tx for mucous membrane pemphigoid (MMP)
refer pt to ophthalmologist for exam and f/u
what is indicated if there is ocular involvement in pts with mucous membrane pemphigoid (MMP)?
systemic imunnisuppressive therapy
T/F: px of mucous membrane pemphigoid (MMP) is rarely fatal
true
T/F: mucous membrane pemphigoid (MMP) can usually be controlled
true
blindness results in mucous membrane pemphigoid (MMP) pts with what?
with untreated ocular disease
T/F: mucous membrane pemphigoid (MMP) sometimes spontaneously resolve
false, rarely undergoes spontaneous resolution
which is the most common of autoimmune blistering conditions?
bullous pemphigoid (BP)
what is the average age of ppl who are affected by bullous pemphigoid (BP)?
older population, average age 75-80 y.o.
where does bullous pemphigoid (BP) primarily affect?
skin
T/F: bullous pemphigoid (BP) primarily appears on skin but can involve the mucous membrane as well
true
what is the gender predilection of bullous pemphigoid (BP)?
no gender predilection
what is an early symptom of bullous pemphigoid (BP)?
pruritus
what follows pruritus in bullous pemphigoid (BP)?
followed by development of multiple, tense bullae, blisters on normal or erythematous skin
T/F: lesions from bullous pemphigoid (BP) rupture, crust and heal with scarring
false, WITHOUT
T/F: oral involvement from bullous pemphigoid (BP) is common
false, uncommon
oral lesions of bullous pemphigoid (BP) rupture sooner than cutaneous lesions leaving what?
large shallow ulcerations with smooth, distinct margins
histo features of bullous pemphigoid (BP)
SUBepithelial cleft similar to MMP
immuno features of bullous pemphigoid (BP)
positive DIF AND IIF with immunoreactants deposited at the BMZ
management of bullous pemphigoid (BP) is similar to cicatricial pemphigoid but most BP cases what?
resolve spontaneously in 1-2 years
T/F: most bullous pemphigoid (BP) pts experience remission
true
why is mortality of bullous pemphigoid (BP) pts 3x higher than age and sex matched population?
problems may develop with use of immunosuppresive therapy in older populations
T/F: etiology of erythema multiforme (EM) is probably immune-mediated
true
immune-mediated condition
immunologic derangement stimulated by trigger that produces that disease
etiology break down of erythema multiforme (EM)
50% - unknown
25% - preceding infection
25% medication-related
25% of erythema multiforme (EM) cases are due to what type of preceding infections?
- viral (herpes)
2. bacterial (mycoplasma pneumoniae)
25% of erythema multiforme (EM) cases are related to what type of medications?
- abx
2. analgesics
who is affected by erythema multiforme (EM)?
young adult, 20-30’s
clinical features of erythema multiforme (EM)
- acute onset ulcerative disorder skin and mucous membranes
- prodromal symptoms ~1 wk before onset
- varying degrees of severity
prodromal symptoms of erythema multiforme (EM)
- fever
- malaise
- headache
- cough
- sore throat
previous studies showed that erythema multiforme (EM) has a male predilection but more recent studies show what?
a female predilection
how long do pts experience prodromal symptoms before onset of erythema multiforme (EM)?
~1 week
varying degrees of severity of erythema multiforme (EM)
- EM minor
2. EM major
EM minor of erythema multiforme (EM) MUCOSA features
- hemorrhagic crusting of vermilion zones
- erythematous patches oral mucosa that undergo necrosis and result in large, shallow erosions and ulcers with irregular borders
which locations are affected by EM minor of erythema multiforme (EM)?
- skin (extremities)
2. mucosa (oral, conjunctival, genitourinary, respiratory)
what mucosa in the oral cavity is usually spared from EM minor of erythema multiforme (EM)?
- gingiva
2. hard palate
EM minor of erythema multiforme (EM) SKIN features
- variety of appearances “multiforme”
- round, dusky-red patches on skin of extremities “target lesions”
- bullae with necrotic centers
EM major of erythema multiforme (EM) clinical features
- 2 or more mucosal sites in conjunction with skin lesions
2. ocular involvement can produce symblepheron
which sites are affected by EM major of erythema multiforme (EM) AND EM minor?
- mucosal
- lip
- skin
different processes of erythema multiforme (EM) than EM major and minor
- Stevens-Johnson syndrome (SJS)
2. toxic epidermal necrolysis (TEN)
Stevens-Johnson syndrome (SJS)
at least two mucosal sites plus skin involvement
toxic epidermal necrolysis (TEN) is also known as what?
Lyell’s disease
toxic epidermal necrolysis (TEN)
diffuse bullous involvement of skin and mucosa
what is the difference between SJS and TEN?
degree of skin involvement and age
SJS has what percent of skin involvement and usually affects who compared to TEN?
<10% of skin invovlement and usually younger pt
TEN has what percent of skin involvement and usually affect who compared to SJS?
> 30% skin involvement and usually over 60 y.o.
distinguishing features between EM and SJS/TEN
- SJS and TEN almost always triggered by a drug, rather than an infection
- SJS and TEN skin lesions begin as red macules or trunk rather than extremities
clinical features of SJS and TEN
- within 2 weeks skin sloughing and flaccid bullae develop
2. pts may appear badly scalded - usually tx’d in burn unit
almost all SJS and TEN pts have what?
mucosal involvement esp oral
dx of EM, SJS and TEN are usually based on what?
clinical presentation
T/F: DIF and IIF are non-specific so it’s not helpful except in ruling out other immune-mediated conditions
true
histo features of EM, SJS, TEN
- subepithelial or intraepithelial vesicles
- necrotic basal keratinocytes
- mixed inflammatory cell infiltrate
- may see perivascular inflammation
supportive therapy for erythema multiforme (EM)
- discontinue causative drug!
- systemic or topical steroids early on
- IV re-hydration
- topic anesthetic or analgesic for pain
tx of SJS and TEN
- discontinue causative drug!
- IV re-hydration
- topical anesthetic or analgesic for pain
what has been implicated in inducing TEN?
NSAIDS
why should steroids be avoided in managing TEN?
associated with increased mortality
how does IV administration of pooled human immunoglobulins seems helpful in resolution of TEN?
by blocking apoptosis of epithelial cells
EM minor and major are self-limiting and should resolve in how many weeks?
2-6 wks
20% of EM minor and major pts get recurrences especially during what time(s)?
spring and autumn
if HSV triggered EM minor or major, what is indicated for maintenance?
antiviral
T/F: erythema multiforme (EM) is not life-threatening except when?
severe cases
mortality rate of SJS
1-5%
mortality rate of TEN
25-30%
px of pts that survives from SJS or TEN
- skin heals in 3-5 wks
- oral lesions take longer
- ocular damage in 50% of pts
erythema migrans is also known as what?
- geographic tongue
2. benign migratory glossitis
etiopathogenesis of erythema migrans is unknown but is probably what?
immune-mediated
erythema migrans is one ofhte more common oral conditions occurring in what percent of the population?
1-3% so considered “common oral lesion”
erythema seen in erythema migrans is due to what?
atrophy of filiform papillae and shearing off of the parakeratin causing epithelium to thin resulting in red appearance
erythema migrans occurs in 1/3rd of pts who have what?
fissured tongue
clinical features of erythema migrans
- wax and wane
- heal then develop in different area
- multiple, well-demarcated zones of erythema surrounded at least partially by slightly elevated yellow-white serpentine or scalloped border
- can be singular “persistens”
where does erythema migrans characteristically affect on the tongue?
dorsal and lateral anterior 2/3 of tongue
ectopic geographic tongue
erythema migrans on other non-keratinized mucosal surfaces
histo of erythema migrans is similar to what?
psoriasis “psoriasiform mucositis” description on bx report
histo of erythema migrans
- parakeratosis with extensive microabscess (neutrophils) formation in superficial spinous layer
- elongation of rete ridges
- neutrophilic infiltration
tx of erythema migrans
none
occassionaly pts with erythema migrans are sensitive or painful to what?
hot, spicy foods… can use potent topical steroid
px of erythema migrans
good
T/F: pts with erythema migrans should be reassured that this is a benign condition
true