Week 2 Flashcards
Types of Pemphigus (4)
-2 ways to GENERALLY differentiate the types (not specifics)
1/2. Vulgaris and Vegetans
3/4. Foliaceus and erythematous
-Generally separate via distinc CLINICAL and MICROSCOPIC presentations.
Which types of pemphigus affect entire epithelium? (2)
Which types affect only upper prickle cell/spinous layer? (2)
Entire epithelium: V! Vulgaris and Vegetans
Prickle/spinous cell: EF! Erythematous and Foliaceus
Review of the hypersensitivities Type I II III IV
- Immediate
- Antibody/antigen AT cells
- Antibody/Antigen in BLOOD (circulating)
- SLOW reaction
What type of ACANTHOSIS is Pemphigus?
- primary or secondary?
- -Example of the other type?
Pemphigus directly attacks the attachment btwn cells
-THUS it is PRIMARY
HSV causes ballooning of the cells and THEN they cause acanthosis
-THUS it is SECONDARY
THIS IS THE ONE THING YOU NEED TO ASSOCIATE WITH PEMPHIGUS FOR BOARDS AND PATH TESTS!
The affected protein is: Desmoglein 3!
Definition of acantholysis?
Loss of intercellular adhesion (proteins)
Strength of Pemphigus Bulla/vesicles?
-Because?
WEAK because they are loss of attachment WITHIN the epithelium.
Pemphigus
DECADE of life
M vs F Ratio?
race differences?
Decade 4th to 5th
M = Female
Ashkenazi Jews
Pemphigus
HLA Phenotypes affected?
-Pneumonic
Dr. 10 dribbles
HLA-DR
HLA-A10
HLA-DRB1
What other diseases are associated with Pemphigus? (5)
-what do they have in common?
They are all autoimmune
- myashenia gravis
- SLE
- RA
- Hashimoto Thyroiditis
- Sjogrens syndrome (venus williams)
Oral lesions of pemphigus look like what other 2 diseases?
Erythema Multiforme
HSV
What % of cases have oral lesions that PRECEDE other cutaneous lesions for pemphigus?
60%
Clinical features of pemphigus 4
Oral lesions
**Nikolsky Sign!
Generalized Bulla/vesicles CHRONICALLY!
4 differentials for Pemphigus?
MMP
Erythema Multi
Aphthous ulcers
Paraneoplastic pemphigus
What differenciates MMP from Pemphigus (4)
- Laminin 5 and BP 180 kd NOT Desmoglein 3
- MMP has occular tissue
- MMP has Low circulating Antibodies
- MMP effects the BMZ
Differences btwn EM and Pemphigus? (3) 1 main one
Target lesions, very acute, etc.
-Type 3 hypersensitivity (EM) not 2
Differences btwn Aphthous ulcers and pemphigus?
Unknown etiology for aphthous ulcers
Difference for paraneoplastic pemphigus and pemphigus?
#1 History of Cancer 2. Large variety of antibodies
Histopath of pemphigus?
- Intraepithelial clefting (indicates acantholysis)
- Tiny Tzank cells (collapsing)
- Basement membrane is intact (loss is within the epithelial layer.
Pathognomic is what?
A characteristic difference only found in one disease!
3 immunologic Vesiculo-bullous (ulcerative) diseases
- Pemphigus Vulgaris PV. 2. Mucous membrane Pemphigoid ((B)MMP) benign. 3. Bullous Pemphigoid
Is the most inferior layer of the epithelium attached to the basement membrane in pemphigus? in a histologic slide
yes, yes it is
Do you use a direct or indirect study on pemphigus?
What 3 antibodies are you looking for?
Direct
looking for IgG, IgA, and C3.
Treatment for pemphigus
- If Skin and mouth=
- If just mouth=
If both, send to dermatologist and they prescribe: monoclonal antibodies (Embrel, etc)
If just mouth then topical steroids!