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1

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.

2

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

3

Review of the hypersensitivities
Type I
II
III
IV

1. Immediate
2. Antibody/antigen AT cells
3. Antibody/Antigen in BLOOD (circulating)
4. SLOW reaction

4

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

5

THIS IS THE ONE THING YOU NEED TO ASSOCIATE WITH PEMPHIGUS FOR BOARDS AND PATH TESTS!

The affected protein is: Desmoglein 3!

6

Definition of acantholysis?

Loss of intercellular adhesion (proteins)

7

Strength of Pemphigus Bulla/vesicles?
-Because?

WEAK because they are loss of attachment WITHIN the epithelium.

8

Pemphigus
DECADE of life
M vs F Ratio?
race differences?

Decade 4th to 5th
M = Female
Ashkenazi Jews

9

Pemphigus
HLA Phenotypes affected?
-Pneumonic

Dr. 10 dribbles

HLA-DR
HLA-A10
HLA-DRB1

10

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)

11

Oral lesions of pemphigus look like what other 2 diseases?

Erythema Multiforme
HSV

12

What % of cases have oral lesions that PRECEDE other cutaneous lesions for pemphigus?

60%

13

Clinical features of pemphigus 4

Oral lesions
****Nikolsky Sign!
Generalized Bulla/vesicles CHRONICALLY!

14

4 differentials for Pemphigus?

MMP
Erythema Multi
Aphthous ulcers
Paraneoplastic pemphigus

15

What differenciates MMP from Pemphigus (4)

1. Laminin 5 and BP 180 kd NOT Desmoglein 3
2. MMP has occular tissue
3. MMP has Low circulating Antibodies
4. MMP effects the BMZ

16

Differences btwn EM and Pemphigus? (3) 1 main one

Target lesions, very acute, etc.
-Type 3 hypersensitivity (EM) not 2

17

Differences btwn Aphthous ulcers and pemphigus?

Unknown etiology for aphthous ulcers

18

Difference for paraneoplastic pemphigus and pemphigus?

#1 History of Cancer
2. Large variety of antibodies

19

Histopath of pemphigus?

1. Intraepithelial clefting (indicates acantholysis)
2. Tiny Tzank cells (collapsing)
3. Basement membrane is intact (loss is within the epithelial layer.

20

Pathognomic is what?

A characteristic difference only found in one disease!

21

3 immunologic Vesiculo-bullous (ulcerative) diseases

1. Pemphigus Vulgaris PV. 2. Mucous membrane Pemphigoid ((B)MMP) benign. 3. Bullous Pemphigoid

22

Is the most inferior layer of the epithelium attached to the basement membrane in pemphigus? in a histologic slide

yes, yes it is

23

Do you use a direct or indirect study on pemphigus?
What 3 antibodies are you looking for?

Direct

looking for IgG, IgA, and C3.

24

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!

25

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)

26

Characteristics of MMP
-Age
-Sex ratio
-Pain?

Age is Adult...
-More women than men!
-Mild to moderate discomfort

27

Characteristics of MMP
Oral lesions ARE (4)

-RED
-ATTACHED gingiva
-Rarely Bullae (vesicles)
-Also rupture easily, but thicker than Pemphigus

28

Characteristics of MMP
Occular lesions (3)

-Scarring of canthus
-inverted lashes
-Cornea trauma (blindness)

29

Characteristics of MMP
Will they be positive for Nikolsky's Sign?

YES

30

Characteristics of MMP
Histopathology

Subepithelial clefting!
(basal layer lifts from BMZ)