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32 Cards in this Set

  • Front
  • Back
What is a blister? (explain the MOA)
Blister is a bubble like space which arises when there is a separation of the the different layers of skin
Pemphigus Vulgaris

Pathogenesis? (MOA)
Autoimmune destruction of desmosomes between keratinocytes
What kind of antibodies are made in Pemphigus vulgaris? Against what structure in the skin?
IgG antibody against the desmosomes (desmoglein = the specific name)
What type of hypersensitivty reaction is pemphigus vulgaris?
Type II

(AB mediated cytotoxic via membrane attack complex)
What is the typical involvment (body part) in pemphigus vulgaris?**

What kind of lesions are formed (note: use derm terminology)
Skin and oral mucosa

Bullae (>1cm and fluid filled)
What are keratinocytes connected by?
Desmosomes

(AB formed against that in Pemphigus vulgaris)
What is Acantholysis?

In what disease does this happen?
Seperation b/w cells (keratinocytes) b/c desmosomes destroyed

happens in pemphigus vulgaris
What layer of the epidermis is the separation formed in pemphigus vulgaris?
Since it is the separation b/w keratinocytes, it seperates the basal layer from the rest of the epidermis

The basal layer keratinocytes are attached to basement membrane via hemidesmosomes (NOT desmosomes)
Nikolsky sign?
The thin walled bullae rupture easily.

When you touch them, their skin easily scrapes off

Pemphigus Vulgaris
What disease is this?
What disease is this?
This is pemphigus vulgaris b/c the basal layer still attached

Tombstone appearance (basal layer)
Immunofluorescence highlighting IgG.  What kind of pattern is this?

Associated with what disease?
Immunofluorescence highlighting IgG. What kind of pattern is this?

Associated with what disease?
Fishnet pattern

Immunofluorescence highlights IgG surrounding the keratinocytes in a fishnet pattern

Pemphigus vulgaris
Bullous Pemphigoid

MOA of disease
autoimmune destruction of hemidesmosomes b/w basal cells and basement membrane
Bullous pemphigoid

What kind of antibodies are produced? Against what?
IgG antibody against basement membrane collagen
What are the common locations to see blisters?
Skin ONLY

(no oral; helps differentiate from pemphigus vulgaris)
How do you differentiate b/w Bullous pemphigoid and pemphigus vulgaris?

(clinically and histology)
BP:
histology - subepidermis blister
clinical - do not rupture easily (milder)

PV:
histology: suprabasal blister
clinically: rupture easily (Nikolsky sign)
What is this?
What is this?
Bullous pemphigoid
Immunofluorescence highlights IgG
Immunofluorescence highlights IgG
IgG along basement membrane

Linear pattern

Bullous pemphigoid
24 yr old male pt. comes in with lesions on his forearm.  When you touch them they don't rupture easily.  What does he likely have?
24 yr old male pt. comes in with lesions on his forearm. When you touch them they don't rupture easily. What does he likely have?
These are blisters (fluid filled; >1cm)

Bullous Pemphigoid
Dermatitis Herpetiformis

MOA of disease
Autoimmune deposition of IgA at the tips of dermal papillae
What is contained in the dermal papillae
many things but blood vessels probably most vulnerable to injury
What kind of skin lesions do you get with Dermatitis Herpetiformis
vesicles (fluid filled <1cm) and bullae (fluid filled >1cm) that are grouped

ITHCY

note: looks like herpes, hence the name
What is Dermatitis Herpetiformis associated with?****
Strong associated with Celiac Disease

resolves with gluten free diet (both)
Dermatitis herpetiformis

grouped fluid filled vessicles/bullae
Itchy
Erythema multiforme

MOA of disease
hypersensitivty reaction characterized by TARGET rash and bullae
Why do you get targetoid appearance in Erythema multiforme?
Central epidermal necrosis leads to the center being white
What is the most common associated with Erythema Multiforme?

Other associations?
Most common: HSV infections

Others: Mycoplasma infection, drugs (eg. penicillin), autoimmune diseases, malignancy
Erythema multiforme
Stevens-Johnson syndrome
EM (target rash) WITH
-mucosa/lip involvment
-Fever
Toxic epidermal necrolysis
Severe form of Steven-johnson syndrome characterized by sloughing of skin, resembling a large burn.

Medical emergency
What is the toxic epidermal necrolysis most often due to?

What is it a severe form of?
most often due to drugs

severe form of Steven Johnson syndrome
What are the common places to lesions for pemphigus vulgaris?**
Skin and Oral mucosa bullae
What is Dermatitis Herpetiformis assocaited with?**
Celiac disease