Immune-mediated Mucocutaneous Disease 1 Flashcards
Which of these mucocutaneous lesions is immune-mediated?
- ectodermal dysplasia
- white sponge nevus
- lichen planus
- Peutz-Jeghers
lichen planus
True or False: With pemphigus vulgaris, the autoantibodies destroy hemidesmosomes.
False, AB destroy desmosomes
True or False: Pemphigus vulgaris has no sex predilection.
True, PV…pemphigus vulgaris…PV…penis or vagina
Pemphigus Vulgaris is a rare immune mediated condition that is _____ if not treated.
fatal
Why is PV fatal when not treated?
SEVERE INFECTION:
- loss of fluids
- malnutrition due to mouth pain
How often do PV patients present with oral lesions? What do oral lesions look like?
50%
superficial, ragged erosions and ulcerations
any mucosal surface
oral blisters seen rarely
PV lesions on the skin appear as _______ _____.
flaccid bullae
nikolsky sign
Describe the presentation of a positive Nikolsky sign.
apply firm, lateral pressure to normal appearing skin
= induces a bulla
True or False: The skin bullae with PV are the “first to show and last to go”
False, the oral lesions are first
oral: initial manifestation and most difficult to resolve
What is immunofluorescence and its sub-categories?
a technique that uses fluorescent-labeled antibodies to detect specific targets
- DIF (direct)
- IFF (indirect)
What is Direct Immunofluorescence used for?
to detect autoantibodies bound to the patient’s tissues
fluoro-IgG + patient biopsy–> microscopy
What is Indirect Immunofluorescence used for?
to detect antibodies circulating in the blood
patient serum + control tissue + fluoro-IgG–> microscopy
True or False: Both direct and indirect immunofluorescence will be negative in pemphigus vulgaris.
False, both DIF and IFF are positive in PV
Normal tissue adjacent to PV ulceration should be sampled for _____.
DIF
Autoantibodies in PV will bind to ______ components.
desmosomal (desmoglein 1 & desmoglein 3)
Where should the PV sample be obtained for biopsy?
Lesions of PV should be sampled at the periphery, no the ulcerated center
What is the microscopic feature that is always present with PV? What is the feature that is usually present?
always: intraepithelial clefting above the basal layer (within the epithelium)
often: acantholysis (breakdown of spinous layer, cells appear to fall apart)
How is Pemphigus Vulgaris treated?
- systemic corticosteroids: often azathioprine or other steroid-sparing agents
- topical corticosteroids have little effect*
True or False: PV is treated with either systemic or topical corticosteroids, whichever they prefer to use.
False, only systemic will work
What is the mortality rate of PV? What was the rate prior to corticosteroid therapy?
5-10% mortality (complications of therapy)
60-90% mortality (prior to meds)
Which immune-mediated mucocutaneous lesion has a similar clinical presentation to PV but is more than 2 times as common?
Mucous Membrane Pemphigoid
Mucous Membrane Pemphigoid is also known as _______ pemphigoid, which means “scarring.”
cicatricial
MMP has a ______ predilection with an average age of _____ years.
female (2:1)
50-60
True or False: MMP may affect any mucosal surface and occasionally the skin.
True
Why is MMP also refered to as Cicatricial Pemphigoid?
scarring is usually seen with conjunctival and cutaneous lesions
(intraoral = rare scarring, may see intact blisters)
Mucous Membrane Pemphigoid often presents as _______ gingivitis. What does this mean?
“desquamative gingivitis”
- erythema, desquamation, ulceration
- seen with several disorders, not just MMP
What is the most significant aspect of MMP?
ocular involvement
- scarring obstructs the orifices of glands that produce the tear film, resulting in dry eye
- dryness leads to keratinization of the corneal epithelium, leading to blindness
For histological purposes, where should the sample of MMP be taken from?
periphery of the lesion, including a generous portion of normal mucosa (epithelium shears off easily)
What are the microscopic characteristics of MMP?
SUBepithelial cleft formation- separation of the epithelium from the connective tissue at the basement membrane
- MMP: between epithelium and CT
- PV: within epithelium
Antibodies in PV will attack the _______; Antibodies in MMP will attack the ______.
PV= desmosomes
MMP= hemidesmosomes
(3 letters = longer word; 2 letters = shorter word)
Oral mucosa submitted for MMP testing should be taken from _______cm away from the areas of ulceration. Tissue should, ideally, be submitted in both ______ solution and _____ solution.
0.5-1.0 cm
Michel’s
formalin
Linear deposition of immunoreactants at the BMZ, with a positive DIF and a negative IFF would indicate that the patient has _____.
mucous membrane pemphigoid
PV: both DIF and IFF are positve
(MMP: only DIF is positive)
What percentage of patients will have circulating autoantibodies against hemidesmosomes (MMP)?
5-25% of MMP patients will have circulating antibodies (which is why IFF is negative)
How is MMPemphigoid treated?
Depends on extent of involvement:
- frequent prophylaxis every 3-4 mos
- oral lesions only = topical steroids, tetracycline/niacinamide or dapsone may be sufficient)
- ocular involvement = systemic immunosuppressive therapy
remember, PV = systemic only! topical has no effect
What is the prognosis of MMP?
rarely fatal, condition is usually controlled
- blindness if ocular disease is left untreated
- rarely resolves spontaneously
True or False: Bullous pemphigoid usually affects a younger population.
False, older
What is the most common initial complaint of bullous pemphigoid? Second most common initial complaint?
- pruritus
2. cutaneous blisters
Which type of lesions are seen most commonly with bullous pemphigoid?
cutaneous
*oral is uncommon: 8-39%
How often are oral lesions found with bullous pemphigoid?
8-39%
True or False: BP Clefting of the epithelium is intraepithelial.
False, BP is suBBBBepithelial (like MMP)
True or False: Both DIF and IFF are positive for bullous pemphigoid.
True
True or False: Most BP cases resolve spontaneously.
True, most within 1-2 years
management is similar to MMP
Erythema Multiforme is an ulcerative disorder that has _____ onset and is self-limiting within _____ weeks.
acute
2-6 weeks
Erythema Multiforme is more likely to affect which of the following:
- 20 year old male
- 20 year old female
- 50 year old male
- 50 year old female
20 year old female
(as of recently. Used to believe that it affected males more, but it is probably immune-mediated so it makes sense that females are more likely)
