17 - Vesiculobullous and immune disease 2 Flashcards

1
Q

What is pemphigoid?

A
  • sub epithelial antibody attack
  • the epithelium and connective tissue split
  • hemidesmosomes are attacked at the basement membrane
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2
Q

How does pemphigoid present?

A
  • thick walled blisters (full epidermis)
  • blister persists
  • can be blood or clear fluid filled
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3
Q

What is bullous pemphigoid?

A

Skin only presentation

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4
Q

What is mucous membrane pemphigoid?

A

All mucous membranes (eyes, mouth, genitals) presentation

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5
Q

What is cicatritial pemphigoid?

A

Mucosal presentation with scarring

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6
Q

What are complications associated with pemphigoid?

A
  • loss of skin barrier leads to infection
  • weeping of fluid leads to dehydration
  • complications of treatment
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7
Q

What are complications associated with pemphigus?

A
  • loss of skin barrier leads to infection
  • weeping of fluid leads to dehydration
  • complications of treatment
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8
Q

How does pemphigoid present with immunofluorescence?

A
  • linear along the basement membrane
  • C3 and IgG are common antigens
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9
Q

What is symblepharon?

A

Scarring of conjunctiva to sclera

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10
Q

How do you manage pemphigoid?

A
  • steroids
  • immune modulating drugs (azathioprine or mycophenolate)
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11
Q

What is pemphigus?

A
  • intraepithelial bullae form due to desmosomes between cells being targeted
  • cells drift way from each other and epithelium thins
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12
Q

How does pemphigus present?

A
  • common sites include mucosa (first) and skin
  • blisters burst quickly so often present with ulceration
  • commonly seen in over 50s, more common in females
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13
Q

How does pemphigus present with immunofluorescence?

A
  • basket weave pattern
  • cells floating between the fluorescence
  • C3 and IgG are common
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14
Q

What is the most common form of pemphigus?

A
  • pemphigus vulgaris
  • usually presents in the mouth before skin
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15
Q

What is the management of pemphigus?

A
  • high dose steroids
  • immunosuppressants
  • biologics
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