Immune-Mediated Mucocutaneous disease Flashcards
Pemphigus Vulgaris is a disease characterized by autoantibodies that destroy ____________
desmosomes
what population groups are most likely to have pemphigus Vulgaris?
- Adults, 4th to 6th decades
- no sex predilection
- Relatively rare
T/F: Pemphigus vulgaris is usually fatal if not treated
True
over ___% of patients with pemphigus vulgaris will present with oral symptoms
50%
what are the clinical characteristics of pemphigus vulgaris?
Ragged erosions and ulcerations
Any oral mucosal surface
Flaccid bullae on skin; intact oral blisters rarely seen
T/F: Oral lesions of pemphigus are often the initial manifestation of the disease and are the first symptoms to resolve after therapy has begun
FALSE
Oral lesions are often the initial manifestation of the disease and the most difficult to resolve with therapy
The “_________ sign” is a clinical hallmark of Pemphigus vulgaris
Nikolsky sign
what do antibodies bind to during Pemphigus vulgaris?
Autoantibodies bind desmosomal components (desmoglein 3 &1)
T/F: during testing for Pemphigus Vulgaris, Both direct (DIF) and indirect (IIF) immunofluorescence studies will be positive
true
what immunoglobulin is used during both indirect and direct immunofluorescence studies?
Fluorescein-labeled, anti-human IgG
what are the histopathological findings of Pemphigus Vulgaris?
1) Intraepithelial (aka suprabasilar) clefting
2) Acantholysis (breakdown of spinous layer) – is also usually evident
3) “Dilapidated brick wall” with INTACT BASAL CELL LAYER
what treatments are used for pemphigus vulgaris?
1) Systemic corticosteroids, often with azathioprine or other steroid-sparing agents
2) Topical corticosteroids have little effect
what is the mortality rate for Pemphigus vulgaris? what was it before the use of systemic corticosteroids?
Prior to corticosteroid therapy, 60-90% mortality
Today, 5-10% mortality, usually due to complications of therapy
For Pemphigus vulgaris, what is the saying that pertains to the oral lesions?
“First to show and last to go.”
“Mucous Membrane Pemphigoid” is also known by what other name?
Also known as cicatricial pemphigoid
cicatricial means “scarring”
MMP will clinically resemble ________ due to blister formation
pemphigus
what population groups are most likely to have Mucous membrane Pemphigoid (MMP)?
A) 2:1 female predilection
B) Older age group – average age, 50-60 years
T/F: Pemphigus Vulgaris is more common than Mucous Membrane Pemphigoid
FALSE
MMP is 2-4 X more common than pemphigus
when is scarring usually seen in MM Pemphigoid?
Scarring usually seen with conjunctival (symblepharon) and cutaneous lesions
What are the clinical characteristics of MM Pemphigoid?
A) May affect any mucosal surface; occasionally skin
B) Often presents as desquamative gingivitis
C) May see intact blisters intraorally
T/F: Scarring from MM pemphigoid is rare intraorally, but is more common with skin (cutaneous) involvement
true
What is the most significant aspect of MM pemphigoids?
Ocular scarring
what causes ocular scarring from MM Pemphigoid?
1) Scarring obstructs the orifices of glands that produce the tear film, resulting in a dry eye
2) Dryness leads to keratinization of the corneal epithelium, leading to blindness
3) Scarring may lead to adhesion formation (symblepharons) between eyelid and globe
list the histopathological characteristics of MM pemphigoid:
Microscopic examination shows:
A) clean subepithelial cleft formation
B) separation of the intact epithelium from the connective tissue at the BMZ
which immunofluorescence study would be positive during MM Pemphigoid? which would be negative?
Positive DIF
IIF usually negative
what epithelial layer is effected during MM Pemphigoid
- the basement membrane zone
- Linear deposition of immunoreactants at the BMZ
Pemphigus effects the _________ of epithelial cells, while Pemphigoids effect the __________
Pemphigus = Desmosomes
Pemphigoids = Hemidesmosomes & BMZ
what are the treatments for MM Pemphigoid?
A) Oral lesions only - topical steroids (first line), tetracycline/niacinamide or dapsone may be sufficient
B) If ocular involvement is present, systemic immunosuppressive therapy is indicated
what is the prognosis for MM Pemphigoid?
1) Rarely fatal
2) Condition can usually be controlled
3) Blindness results in patients with untreated ocular disease
4) Rarely undergoes spontaneous resolution
in what population groups are Bullous Pemphigoid most commonly found?
Usually older adults, 75-80 yrs
T/F: oral involvement is common in Bullous Pemphigoid
FALSE
only 10-20% will have oral involvement
what are the initial symptoms of Bullous Pemphigoid?
Pruritus (itching) is common initial complaint, followed by cutaneous blisters
how do Bullous Pemphigoid and MM Pemphigoid differ when doing a immunofluorescence study?
Both will have Subepithelial clefts
A) For Bullous Pemphigoid- Positive DIF and IIF
B) For MM pemphigoid- only the DIF is positive
T/F: Like MM Pemphigoid, Bullous Pemphigoid effects the basement membrane zone of epithelial cells
True
how is Bullous Pemphigoid treated?
Management similar to MM pemphigoid, but immunosuppressive therapy can have serious side effects
Many cases resolve spontaneously in 1-2 years
what histopath characteristic is seen in both Bullous and MM Pemphigoid?
Subepithelial clefts
_______________ is an ACUTE, self-limiting ulcerative disorder
Erythema Multiforme
what is the Etiology of Erythema Multiforme?
- 50% - unknown
- 50% - most are infection-related (often HSV) or (less commonly) medication-related
who is most likely to acquire Erythema Multiforme?
Young adult females
what are the 3 forms of Erythema Multiforme?
A) EM minor
B) EM major
C) Stevens-Johnson syndrome & toxic epidermal necrolysis
how does EM minor differ from EM major?
EM minor – skin or oral mucosa only
EM major – at least TWO mucosal sites PLUS skin involvement
what is the difference between Stevens-Johnson syndrome & toxic epidermal necrolysis?
(reminder, they are both forms of Erythema Multiforme)
Stevens-Johnson: less than 10% of body involved
TEN: over 30% of body is involved
What are the Clinical characteristics of Erythema Multiforme?
** A) Hemorrhagic crusting of lips **
B) Widespread oral ulcers with ragged margins
C) “target” lesions of skin
what oral sites are commonly effected during Erythema Multiforme?
Labial mucosa, buccal mucosa and tongue
T/F: the DIF results for Erythema Multiforme will be non-specific
True
how is Erythema Multiforme treated?
give treatment for EM major, minor, and Toxic epidermal necrolysis
1) Supportive care (analgesics, soft diet, hydration) for mild cases
2) Somewhat controversial for EM major – corticosteroids are often given empirically
3) TEN managed in burn unit; IV pooled human immunoglobulin shows promise
what is the PROGNOSIS for Erythema Multiforme?
give prognosis for EM major, minor, and Toxic epidermal necrolysis
Good for mild to moderate cases
EM major – 1-5% mortality
TEN – 25-30% historically; IV Ig therapy has dramatically improved patient recovery
what is the recurrence rate for Erythema Multiforme?
WHEN is recurrence most likely?
EM may be recurrent in 20% of cases
– usually autumn & spring
“benign migratory glossitis” is a form of what disease?
Erythema migrans
T/F: Benign Migratory Glossitis is one of the most common oral conditions
True
One of the more common oral conditions, occurring in 1-3% of the population
benign migratory glossitis (erythema migrans) is also known as “______________”
geographic tongue
what are the clinical characteristics of benign migratory glossitis (erythema migrans)?
A) Often seen with fissured tongue
B) Patients typically report waxing and waning of lesions
C) May develop on other non-keratinized mucosal surfaces
what is the term given for benign migratory glossitis developing on NON-keratinized mucosal surfaces other than the tongue?
“ectopic geographic tongue”
T/F: the histopathology for benign migratory glossitis is similar to psoriasis
true
biopsies are often diagnosed as “psoriasiform mucositis”
what are the histopath characteristics of benign migratory glossitis?
Parakeratosis with extensive microabscess formation in the UPPER SPINOUS layer
- Results in LOSS of SUPERFICIAL PARAKERATIN
- Remaining epithelium is much thinner and appears red
what is the treatment protocol for Benign Migratory Glossitis?
A) No treatment is generally necessary
B) Some patients may complain of sensitivity to hot or spicy foods when lesions are active
C) May respond to one of the stronger topical corticosteroid gel preparations
what are the microscopic characteristics of Erythema Multiforme lesions?
1) Keratinocyte destruction
2) sub epithelial edema
3) mixed inflammatory infiltrate
4) perivascular inflammation
