Vesiculobullous Disease 2 Flashcards

1
Q

Pemphigoid

A
  • relatively common
  • sub epithelial antibody attack
  • thick walled blisters (full epidermis)
  • usually persist to be seen
  • clear/ blood filled blisters
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2
Q

Different forms and presentation of pemphigoid

A
  1. Bullous pemphigoid - skin
  2. Mucous membrane pemphigoid
    - all mucous membrane
    - eye, genital, oral
  3. Cicatritial pemphigoid
    - mucosal with scarring
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3
Q

Histopathology of pemphigoid

A
  • sub epithelial split at epithelial/ CT junction
  • Hemi- desmosomes involved at basement membrane
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4
Q

Clinical appearance of Pemphigoid on skin

A
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5
Q

Immunofluorescence of Pemphigoid

A
  • linear staining along basement membrane
  • C3 and IgG detected in this area in standard pemphigoid
  • IgA sometimes found

*Linear staining with C3 is called “Linear IgA disease”
*Granular IgA and C3 deposits is seen in Dermatitis Herpetiformis

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6
Q

Pemphigoid- Symblepharon

A
  • scarring of conjunctiva which bind eye surface of eyelid
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7
Q

Where can you normally pemphigoid?

A

Oral & skin lesions
- both can cause lesion
- bullous usually involves skin
- mucous membrane usually mouth/ eye/ genital
(need to involve specialist)

Scarring
- scarring when healing in Cicatritial pemphigoid

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8
Q

What happen after healing for Cicatritial pemphigoid?

A

scarring

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9
Q

Management of Pemphigoid

A
  1. Steroid
  2. Immune modulating drug
    - Azathioprine
    - Mycophenolate
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10
Q

About pemphigus

A
  • commonest is Pemphigus vulgaris
  • intraepithelial bullae

Clinical
- uncommon before age 50
- Female > male
- genetic - Ashkenazi Jews

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11
Q

Which sites may cause Pemphigus

A
  • skin
  • mucosa - often first site
  • both eventually involved
  • blisters - burst - spread
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12
Q

Histopathology of Pemphigus

A
  • supra-basal split, only desmosome involved
  • tzank cells
  • basket- weave pattern
  • intraepithelial
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13
Q

Immunofluorescence for Pemphigus

A
  • basket- weave staining around each of epithelial cells
  • C3 and IgG in Pemphigus Vulgaris
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14
Q

Clinical photos of pemphigus

A
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15
Q

More photos Pemphigus

A
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16
Q

About pemphigus

A

Pemphigus Vulgaris
- affects mucosa and skin
- oral lesions before skin

Rarely see intact bullae
- intra-epithelial blister
- surface easily lost

17
Q

Management of Pemphigus Vulgaris

A
  • fatal without tx
  • often complications of tx are major cause of death
18
Q

Summary of Vesiculobullous disorders

A
  • uncommon in primary care
  • Pemphigoid is least common (consider this when pt reports blistering disease)
  • conditions can be fatal and should be referred to specialist
19
Q

Pemphigus

A

Anti- desmoglein 1/3 antibody

20
Q

Nikolsky’s sign

A
  • +ve in Pemphigus
  • -ve in Pemphigoid
21
Q

Protein in Hemidesmosome

A

BP180
BP 230

22
Q

ELISA used for

A
  • test antibodies