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
5 Differentials for MMP
Discoid Lupus, what is the diff?
Butterfly rash on malar bones
Treatment for MMP,
what is the cure?
THERE IS NO CURE!!!!! only managed
Treatment for MMP
Refer to (2)
Treatment (3)
Refer to rheumatologist AND ophthalmologist.
Treat with: steroids, Tetracycline w/ niacinamide, and Monoclonal Antibodies.
Bullous Pemphigoid characteristics -Age -Antibodies types -Antibody levels
Age 70-80
Antibody types: Laminin, BP 180 kd, AND BP 230 kd.
Antibody levels: High enough for an indirect test.
BP
Characteristics
-lesion location (2)
- Skin
- Oral
NOT EYES NOT EYES!
MMP has eye involvement
Epidermolysis Bullosa
Types 2
Acquired
Hereditary/Genetic = Dystrophic
EB aquired form characteristics -A.K.A. -Antibodies (2) -How it is acquired?
Aka-epidermolysis acquisita
Antibodies: IgG @ BMZ, and Type VII collagen BELOW Lamina densa
-Acquired via drugs
EB
Genetic/hereditary/Dystrophic/junctional/simplex
-TWO characteristics
Enamel pitting
No circulating antibodies! (no indirect testing allowed)
EB
Clinical findings Oral: (3)
- Scarring bulla
- Hypoplastic TEETH
- Limited mouth openin (due to scarring)
EB clinical findings (non-oral)
2
- Bulla formation from
- trauma
- Stress - Dystrophic Nail beds
Treating EB (2)
- Steroids
- Vitamin E
Ulcerative Conditions
-Definition of ulcer
-Discontinuation of the epithelium DUE TO death of cells
Ulcerative conditions
-Key trait of malignant ulcers?
PAINLESS! very important for early detection
Ulcerative conditions
-Key characteristic of self-mutilating ulcers
Linear ulceration with chosen traumatic instrument
Where do you find traumatic ulcers most and 2nd most often?
- Tongue - DRINKS
2. Hard Palate - PIZZA BURN
FACTITIAL INJURY means what?
Self induced
Clinical features of Traumatic Ulcers (TU)
- Acute vs Chronic (2)
- —Pain, Margin,
Acute Pain- Painful
Chronic Pain - No pain
Acute Margin - “yellow base” Red margin
Chronic margin - “yellow base” White margin
Four types of Acute ulcers
Thermal chemical mechanical aphthous
The ONE thing ALWAYS on your list of differentials, if a chronic ulcer presents?
SCC
-Squamous cell carcinoma
What type of necrosis will happen from topical aspirin?
Coagulative
What causes most chemical ulcerations?
H2O2 (teeth bleaching)
WHere do you find radiation ulcers vs chemo ulcers?
Radiation is at the therapy location
Chemo can be found anywhere!
Histopathology of Trauma ulcers (2)
Fibrinous exudate
Fibrin network with PMN’s
What does TUGSE stand for?
Traumatic Ulcerative Granuloma w/ stromal eosinophilia
histopathology of TUGSE what cells are found? (5)
- Eosinophils (in C.T.)
- Lymphocytes (this is a more chronic ulcer)
- Nphil
- Macrophage
- Plasma cells
- Lymphocytes (this is a more chronic ulcer)
Recall time for an ulcer (concern about it being chronic?)
2 WEEKS
Bacteria that causes Syphilis
Treponema Pallidum
What Symptom is seen in Syphilis?
-Primary
Chancre
-Ulcer AT SITE OF Infectious transfer
What Symptom is seen in Syphilis?
-Secondary (3)
NOT ULCERS
- Mucous patches
- Condyloma Latum
- Maculopapular Rash
What Symptom is seen in Syphilis?
-Tertiary (3) Neurosyphilis and Cardiovascular
- Gumma
- Mucosal Atrophy
- Palatal Perforation
Definition of Granuloma (5)
Collection of epitheliod Macrophages
- Lymphocytes
- Histocytes
- Foreign Body Giant cells OR Langerhans Giant cells (horseshoe shape)
- —Necrosis in the center
symptoms of congenital Syphilis (4)
HUTCHINSON TRIAD
- Interstitial keratitis
- VIII deafness
- TAPERING Incisors
- Mulberry molars
Histopathology of Primary and Secondary Syphilis (2)
Proliferative endarteritis
*Plasma cells
Proliferative endarteritis is what?
Inflammation around B.V.
-Causing proliferation of tunica Intima and occluding the vessels.
What is a Gumma? (2)
Granulomatous lesions
-Necrosis
Differentials of Primary Syphilis (2)
SCC Chronic ulcers (traumatic, i guess...)
Differential for secondary syphilis? *****
NOT ULCERS
Differential for tertiary syph? (5)
- T-Cell Lymphoma
- Deep fungal infection
- Salivary gland neoplasm
- Scc
- Cocaine
Treatment for Syph?
Penicillin G
What (via a Serological test) may we get a pos. rxn for syph?
SLE.
Gonorrhea what symptoms?(2)
Pharyngeal ulcerations
-Cervical lymphadenopathy
Differential for Gonorrhea (4)
Aphthous ulcer
Herpetic ulcers
Streptococcal infection
EM
TB bacteria
Mycobacterium Tuberculosis
Spread via (2)
AIRBORNE
-Implantation in any cuts or openings in your mouth
****The bacteria does not cause the lesions, it is caused by the bodies reaction (type 4 HS) and inability to eradicate the Bugs
BLANK
Histopathology of TB (2)
Caseous Necrosis
-Granulomas
Actinomycosis
- Gram
- Aerobicity?
Gram pos
Anaerobic
Actinomycosis
-Is this always a pathogen?
NOPE
-commensalism organism
Actinomycosis
-Symptoms (3)
- Numbs lip (swelling)
- Painful (swelling) Osteomyelitis
- Fistula (Sulfur granules, colored by CFU)
Actinomycosis
-4 things cause numbness DIFFERENTIAL
- Stroke
- Trauma
- Tumor
- Osteomyelitis
Actinomycosis
- Two other microbes that cause osteomyleitis?
Bact - TB / Staph
(fungal can cause it too)
Actinomycosis treatment
Penicillin
Actinomycosis Histopath (2)
Bacterial colonies
—w/ Nphils
Granulation tissue (chronic lesion)
What carries histoplasmosis (source)
FLYING POOPERS
-pigeons and Bats
Symptoms of Deep fungal infections (4)
Lung invoved -Chronic ulcer -Undermined border ***Peripherally LUMPY (Looks like malignancy)
Who do you find fungal infections in? (2)
Immunosuppressed (including diabetics)
4 Differentials for Deep fungal
- Traumatic ulcers
- TB (oral lesions)
- Primary Syph
- SCC
Histopath (2) deep fungal
- Fake epithelial hyperplasia
2. Granuloma
People who get Opportunistic fungal infections
always found on the body
- Diabetic
- Malignancies (adv.)
- Radiation
- Immunosuppressed
