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DS4101 > W13 - Vesiculo Bullous Disorders - Thomson > Flashcards

W13 - Vesiculo Bullous Disorders - Thomson Flashcards

1
Q

What is a vesiculobullous disorder?

A

Chronic inflammatory disorder

  • Auto antibodies target structural proteins in desmosomal plaques in skin/mucosa
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2
Q

How do VB disorders appear clinically?

A

Vesicles / bullae

intra or sub epithelial blistering

erythema

erosions/ulsers

Pain, foetor

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3
Q

Mucous membrane pemphigoid (features, what is it, histopathology)

A
  • most common vbd
  • Scar formation - oesophageal, laryngeal, conjuctival
  • sub epithelial blistering
  • Loss of connection btw basal epithlial cells and dermis
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4
Q

Most common VBD

A

Mucous membrane pemphigoid

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5
Q

Etiology of mucous membrane pemphigoid

A

•IgG and/or IgA Auto-Antibodies target Hemi-Desmosomes in Epithelial Basement Membrane Zone (EBMZ)

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6
Q

Clinical variants of mucous membrane pemphigoid (4)

A

Desquamative gingivitis

Erythematous & hyperaemic

Small bullae formation

Extends beyond marignal gingiva

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7
Q

Clinical features of mucous membrane pemphigoid

A
  • 55-65 yo
  • Thick-walled bullous lesions
  • Lasts several days
  • Irregular erosions/ulcers after bullae burst
  • Oral lesions heal without scarring
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8
Q

ID and diff dx

A

Mucous membrane pemphigoid

  • large ulceration after bullae burst
    • How to differ from erythroleukoplakia?
  • “Creamy” looking lesion NOT white
  • Flat, not raised
  • looks more like blister/ulcer
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9
Q

How to diagnose mucous membrane pemphigoid

A

Take incisional biopsy from around the tissue

  • “perilesional sample”
  • NOT from the centre → will just crumble
  • Take 2 sample
    • One goes into 10% saline solution
    • One becomes frozen sample
  • 3:38 → listen again
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10
Q
A

Mucous membrane pemphigoid

  • Epithelium detaches from underlying lamina propria
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11
Q

Whats going on here

A

Glowing effetct - autoantibody attacking membrane basement cell junction

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12
Q

Examples of direct immunofluorescence

A

Patient’s biopsy sample

Frozen

Section

Microscopy

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13
Q

Examples of indirect immunofluorescence

A

Patient’s Serum

Substrate - salt split skin

Microscopy

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14
Q

Whats going on here

A

linear igG and C3 along epithelial basement membrane zone

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15
Q

Tx of mucous membrane pemphigoid

A
  • Refer to opthamology and derm
  • Topical corticosteroids
  • Systemic corticosteroids (prednisone)
  • Immunosuppressant therapy
  • Maintenance of OH
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16
Q
A

Pemphigus vulgaris

“a vulgar disease”

worse than mm pemphigoid

  • potentially lethal
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17
Q

Features of pemphigus vulgaris

A
  • Potentially lethal
  • Skin and oral mucosa affected
  • chronic
  • Intra-epithlial blistering
  • Can be drug induced
18
Q

Cellular etiology of pemphigus vulgaris

A

IgG autoantibody binding targets desmosomal proteins interfering with cell adhesion

19
Q

Clinical appearance of pemphigus vulgaris (3)

A
  • Painful, fragile, fluid-filled blisters
  • Burst in a few hours
  • shallow ulcers/ erosions
20
Q

What tissues are affected by pemphigus vulgaris

A

Affects both palates, buccal mucosa, lips and gingiva

21
Q
A

Pemphigus vulgaris

22
Q

ID and whats going on

A

IgG, IgM and C3 bound to intercellular areas of epithelium

  • – Fluorescently-labelled Antibodies to identify bound Auto-Antibodies
23
Q

What is the treatment of pemphigus vulgaris

A
  • Refer to dermatologist
  • Systemic corticosteroids (prednisone)
  • immunosuppressive therapy
  • Topical corticosteroids
  • Maintenance of OH
24
Q

Diff dx of mm pemphigoid and pempigus vulgaris (4)

A
  • Paraneoplastic Pemphigus (Haematological Malignancies)•
  • Erythema Multiforme•
  • Epidermolysis Bullosa•
  • Angina Bullosa Haemorrhagica
25
Q

What can be used to diagnose VB disorders (3)

A

Clinical feats

Biopsy samples

serum samples

26
Q
A

Erythema Multiforme

  • “blood stained crusting”
27
Q

Describe the range of erythema multiformw

A

EM minor - skin (arms)

EM major - 2 mucosal sites

Stevens-johnson syndromes - 10% of body surface area

Toxic epidermal necrolysis - >30% body surface area, lethal

28
Q

Tx of erythema multiforme (3)

A

Infectious / drug trigger (remove)

Systemic corticosteroid therapy

Supportive measures

29
Q

Tx of erythema multiforme (3)

A

Infectious / drug trigger (remove)

Systemic corticosteroid therapy

Supportive measures

30
Q
A

Erythema multiforme

31
Q
A

angina bullosa haemorrahagica

32
Q
A

angina bullosa haemorrahagica

33
Q
A

angina bullosa haemorrahagica

34
Q

Features of angina bullosa haemorrahagica (3)

A
  • common
  • most commonly on soft palate and esophageal area
  • (can also be on tongue or buccal mucosa)
  • feels like “choking/strangulation”
35
Q
36
Q
A

MM Pemphigoid

37
Q
A

Pemphigus vulgaris

38
Q
A

Paraneoplastic pemphigus

40
Q
A

MM pemphigoid

41
Q

How to differentiate between pemphus vulgaris and erythema multiforme

A

Erythema multiforme is more rare but will present in response to a virus or drug or something

  • must consider the other pt history (ex. immunofluoresence)

DS4101 (43 decks)

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