Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Prof Thomson > Week 13- Vesicullo Bullous Disorders > Flashcards

Week 13- Vesicullo Bullous Disorders Flashcards

1
Q

What are signs and symptoms of vesicullobullous disorders?

A
  • Vesicles or Bullae
  • Intra-Epithelial or Sub-Epithelial Blistering
  • Erythema, Blisters, Erosions, Ulcers
  • Pain, Dysphagia, Foetor
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are 2 common vesicullobullous disorders?

A

Pemphigus vulgaris

Mucous membrane pemphigoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is a vesiculobullous disroder?

A
  • Chronic Inflammatory Disorder
  • Auto-Antibodies target Structural Proteins of Desmosomal & Hemi-Desmosomal Plaques in Skin & Mucosa
  • Formation of vesicles or bullae
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is this?

A

Mucous Membrane Pemphigoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the most common vesiculobullous disorder?

A

Mucous membrane pemphigoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the pathophysiology of mucous membrane pemphigoid?

A
  • IgG &/or IgA Auto-Antibodies target Hemi-Desmosomes in Epithelial Basement Membrane Zone
  • Loss of Connection between Basal Epithelial Cells & Dermis
  • Sub-Epithelial Blistering
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are characteristics of mucous membrane pemphigoid?

A
  • Thick-walled bullous lesions burst & form ulcers
  • Last Several Days before Ulceration
  • Oral Lesions usually Heal without Scarring
  • Non-Keratinized Mucosa
  • May be Blood-Filled
  • Desquamative Gingivitis
  • Erythematous & Hyperaemic
  • Extends beyond Marginal Gingiva
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What age is mucous membrane pemphigoid common and how long does it last?

A

Last Several Days

55-65 Years of Age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are histopathological features of mucous membrane pemphigoid diagnosis from incisional biopsy?

A
  • Perilesional sample
  • Subepithelial bullae
  • No acantholytic cells
  • Epithelium detaches from underlying lamina propria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the glowing effect in this sample?

A

Autoantibody attacking membrane basement cell junction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are 2 types of immunofluorescence?

A
  • Direct (biopsy sample): frozen, section, microscopy
  • Indirect (pts serum): serum, substrate (salt-split skin), microscopy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is tx for mucous membrane pemphigoid?

A
  • Refer for specialist care
  • Topical or systemic corticosteroids
  • Immunosuppressant therapy
  • Maintenance of OH
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is this?

A

Pemphigus vulgaris

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are characteristics of pemphigus vulgaris?

A
  • Potentially lethal- infection, fluid & protein loss, dysphagia
  • Oral lesions precede skin
  • Rare
  • Chronic
  • Can be drug induced
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is pathophysiology of pemphigus vulgaris?

A
  1. IgG Auto-Antibody Binding targets Desmosomal Proteins interfering with Cell Adhesion
  2. Acantholysis
  3. Intra-Epithelial Blistering
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are clinical features of pemphigus vulgaris?

A
  • Painful, Fragile, Fluid-Filled Blisters
  • Soft / Hard Palate, Buccal Mucosa, Lips & Gingivae
  • Burst within Few Hours
  • Shallow Ulcers / Erosions
17
Q

What is the histopathology for pemphigus vulgaris following incisional biopsy?

A
  • Perilesional Sample
  • Acantholysis
  • Intra-Epithelial Bullae
  • Leucocytic Infiltrate in Lamina Propria
18
Q

What does immunofluorescence indicate for pemphigus vulgaris?

A

IgG, IgM & C3 bound to Intercellular areas of Epithelium

19
Q

What are the 2 biopsy samples used for?

A

Histopatholgy

Direct IMF microscpy

20
Q

What can be used to diagnose vesiculubullous disorders?

A

Clinical features

Biopsy samples

Serum samples

21
Q

What are other vesiculobullous disorders?

A
  • Paraneoplastic Pemphigus (Haematological Malignancies)
  • Erythema Multiforme
  • Epidermolysis Bullosa
  • Angina Bullosa Haemorrhagica
22
Q

How is pemphigus vulgaris treated?

A
  • Refer for specialist care
  • Systemic corticosteroids (prednisolone 40-60mg daily)
  • Immunosuppressive therapy
  • Topical corticosteroids
  • Maintenance of OH
23
Q

What are immunofluorescent signs of mucous membrane pemphigoid?

A

Linear IgG & C3 along Epithelial Basement Membrane Zone

24
Q

What are signs of erythemia multiforme?

A

Oral/labial erythema, blistering and ulceration

25
Q

What’s the difference between erythema multiforme minor and major?

A
  • EM minor: skin/target lesions
  • EM major: cutaneous + 2 mucous sites
26
Q

What is angina bullosa haemorrhagica?

A
  • Not uncommon
  • Blood blisters on soft palate and pharynx mostly
  • Can be painful
27
Q

What is this?

A

Angina bullosa haemorrhagica

28
Q

What are differential diagnoses for vesiculobullous disorders?

A
  • Mucous membrane pemphigoid
  • Pemphigus vulgaris
  • Paraneoplastic pemphigus
  • Stevens-Johnson syndrome
  • Oral lichen planus
29
Q

What is tx for erythema multiforme?

A
  • Determine infectious/drug trigger
  • Systemic corticosteroid therapy
  • Supportive measures
30
Q

Where is the cleft in pemphigoid vs pemphigus?

A
  • Mucous membrane pemphigoid: subepithelial cleft
  • Pemphigus vulgaris: intraepithelial cleft
31
Q

What % body area is affected in stevens-johnsons syndrome?

A

10%

32
Q

What is this?

A

Erythema multiforme

33
Q

What is this?

A

Erythema multiforme

34
Q

What is this?

A

Mucous membrane pemphigoid

35
Q

How do you differentiate between pemphigus vulgaris and erythema multiforme?

A
  • Immunofluorescence
  • Erythema multiforme is often associated with viral infection or drugs
36
Q

Where can scar formation occur with mucous membrane pemphigoid?

A

Oesophus, Larynx, Conjunctiva

37
Q

What is a subtype of mucous membrane pemphigoid?

A

Linear IgA Disease (LAD) - Subtype

Prof Thomson (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions