Flashcards in Immune-Mediated Mucocutaneous disease Deck (57)
Pemphigus Vulgaris is a disease characterized by autoantibodies that destroy ____________
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
over ___% of patients with pemphigus vulgaris will present with oral symptoms
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
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
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
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
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
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
What is the most significant aspect of MM pemphigoids?
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?
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