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!
Characteristics of MMP
- Antigens affected (2)
- Antigen levels?
- Tissues affected (2)
- Antigens: BP 180 kd and Laminin 5
- Low levels of circulating antibodies
- Tissues affected: Eyes and Oral (skin)
Characteristics of MMP
- Age
- Sex ratio
- Pain?
Age is Adult…
- More women than men!
- Mild to moderate discomfort
Characteristics of MMP
Oral lesions ARE (4)
- RED
- ATTACHED gingiva
- Rarely Bullae (vesicles)
- Also rupture easily, but thicker than Pemphigus
Characteristics of MMP Occular lesions (3)
- Scarring of canthus
- inverted lashes
- Cornea trauma (blindness)
Characteristics of MMP
Will they be positive for Nikolsky’s Sign?
YES
Characteristics of MMP
Histopathology
Subepithelial clefting!
basal layer lifts from BMZ
Immunoflourescent testing has two types what are they?
Direct and indirect
Direct definition
Requires Biopsy/Patients tissue
-exposed to antibodies labeled with fluorescence.
Indirect definition
Take patients blood (use the serum) and tag it with the color and place that on a different host’s tissue (rat bladder)
Which diseases use Direct fluoroscopy? (2)
Pemphigus Vulgaris
MMP
Which disease uses Indirect?
Paraneoplastic pemphigus (many different antibodies looked for)
**NOT for MMP, it has LOW circulating antibodies
5 Differentials for MMP
Pneumonic
- Pemphigus V
- Lichen planus
- Linear IgA
- Discoid Lupus erythematosus
- Lichenoid mucous
5 Differentials for MMP
How to differenciate?
Take biopsy and Direct Immunofluorescent test!
5 Differentials for MMP
Why not Linear IgA?
There is an IgA linear border at the BMZ