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Oral candidiasis > 13. Vesiculobullous diseases > Flashcards

13. Vesiculobullous diseases Flashcards

1
Q

What is an intraepithelial vesicle?

A

A vesicle within the epithelium

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2
Q

What are the 2 main types of intraepithelial vesicles?

A

Acantholytic
Non-acantholytic

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3
Q

What is the definition of acantholytic vesicle?

A

Intraepithelial
Break down of the intercellular attachment that actually holds the epithelial cells attached to each other (destruction of prickle cell layer).

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4
Q

What diseases is acantholytic vesicles seen in?

A

Pemphigus
Darier’s disease
Benign familiar pemphigus- Hailey Hailey Disease

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5
Q

What are non-acantholytic vesicles due to?

A

Death and rupture of epithelial cells

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6
Q

What diseases is non-acantholytic vesicles seen in?

A

Herpes Simplex
Herpes Zoster
Coxsackie A viruses

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7
Q

What is subepithelial vesicle?

A

Between the epithelium and lamina propria of connective tissue, so the roof of the vesicle is epithelial tissue and bottom of the vesicle is connective tissue.

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8
Q

What are examples of subepithelial vesicle diseases?

A

Epidermolysis bullosa
Pemphigoid
Bullos lichen planus
Dermatitis herpetiform
Linear IgA disease
Angina bullous haemorrhagica
Erythema multiforme

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9
Q

What are the 4 main mechanisms of vesicle formation?

A

Infective- death and rupture of intraepithelial cells
Immunological
Traumatic
Idiopathic

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10
Q

What are 3 examples of immunological?

A

Pemphigus
Pemphigoid
Epidermolysis bullosa acquisita

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11
Q

What are 2 examples of traumatic vesicle formation?

A

Epidermolysis bullosa
Angina bullosa haemorrhagica

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12
Q

What are 3 examples of idiopathic vesicle formation?

A

Erythema multiforme
Lichen planus
Angina bullous haemorrhagica

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13
Q

What is pemphigus?

A

Autoimmune reaction against stratified suprabasal epithelium adhesions proteins.

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14
Q

What antibodies are produced in pemphigus vulgaris?

A

Anti-desmoglein 3 antibodies

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15
Q

What antibodies are produced in pemphigus foliaceous?

A

Anti-desmoglein 1 antibodies

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16
Q

What antibodies are produced in peraneoplastic?

A

Anti desmoplakin 1 and 2 Antibodies or IgA or IgG

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17
Q

What antibodies are produced in IgA pemphigus?

A

Anti-desmocollin 1, 2 and 3 antibodies or IgA

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18
Q

What are the 6 clinical types of pemphigus?

A

Pemphigus vulgaris- rarely in mouth
Paraneoplastic
IgA pemphigus
Pemphigus follaceous- less serious, rarely in mouth
Pemphigus vegetans
Pemphigus erythematosus

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19
Q

Why is pemphigus fatal if untreated?

A

Due to extensive ulceration, electrolyte loss and infection

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20
Q

Where is pemphigus often seen first?

A

Mouth

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21
Q

What is Nikolksy’s sign?

A

Pinch the skin of a patient with pemphigus and it should leave a vesicle. If you gently press the vesicle, it will spread laterally. But do not do this, instead look at the rubbed surfaces such as the arm or mouth.

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22
Q

What are cells attached by?

A

Desmosomes- desmoglein and desmocollins. They are found in the extracellular core region.

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23
Q

What is inside the cells that help attachment?

A

Attachment plaques and desmoplakin, and plakoglobins.

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24
Q

What does the attachment plaque attach to inside the cells?

A

Keratin intermediate filaments

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25
Q

What do the vesicles in pemphigus appear like?

A

Thin-roofed vesicles/bullae followed by ulceration
They are intraepithelial, acantholytic lesions, suprabasal spliit

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26
Q

What can be found in the blister fluid in pemphigus?

A

Tzanck cells
They are swollen, and have a hyperchromatic nucleus.
They are epithelial cells that do not have anywhere to attach so they float about in the fluid.

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27
Q

What immunofluorence can be used for pemphigus?

A

IgG and C3 intercellularly

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28
Q

What are the 5 histology factors of pemphigus?

A
  1. Thin-roofed vesicles/bullae (acantholytic, intraepithelial, suprabasal split) followed by ulceration
  2. Tzanck cells in the blister fluid- swollen, hyperchromatic nucleus
  3. Little inflammatory infiltration until ulceration
  4. Basal cells remain attached- tombstone appearance
  5. Immunofluorescence- IgG and C3 intercellularly
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29
Q

How are basal cells attached?

A

Attached to one another by desmosomes and to the cells above them
Attached to underlying basement membrane by hemidesmosomes.

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30
Q

What is the direct method of immunofluorescence for pemphigus?

A

Biopsy incubated with fluroscein-conjugated anti-human IgG and anti-c3 sera

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31
Q

What is the indirect method of immunofluorescence for pemphigus?

A

Patient serum incubated on normal animal mucosa, then labelled with fluoroscein-conjugated anti human globulin.

32
Q

What is the treatment for pemphigus?

A

Systemic corticosteroids
Steroid sparing agents
Immunosuppressive agents

33
Q

What is Benign familial pemphigus?

A

Intraepithelial-acantholytic
Rare genetic autosomal dominant dermatologic disease

34
Q

What gene does benign familial pemphigus affect?

A

ATP2C1 gene on chromosome 3
Codes for SPCA1- secretory pathway Ca/Mn-ATPase
This is a calcium and magnesium pump. Calcium is necessary for binding of desmosomes.

35
Q

What is the clinical signs of benign familial pemphigus?

A

First signs appear from 15-40
Nikolsky positive
Red scaly areas or small blisters at areas of friction
Oral lesions similar to skin

36
Q

What are the histology and immunofluorescence findings of benign familial pemphigus?

A

Intraepithelial acantholytic
Histologically similar to pemphigus- collapsing brick wall
Immunological findings are negative

37
Q

What is Darier’s disease/keratosis follicularis, and what gene does it affect?

A

Autosomal dominant
ATP2A2 gene
Abnormal desmosome-keratin filament complex

38
Q

What is the clinical presentation of Darrier’s?

A

Multiple pruritic, hyperkeratotic papules and plaques in head, neck and trunk
Palmar/sole pits
Nail dystrophy
Oral lesions
Peak incidence 11-40 years old

39
Q

What is the histology of Darrier’s?

A

Intraepithelial, acantholytic blisters
Corp ronds- round nuclei with perinuclear halo
Corp grains- elongated grain shaped nucleus

40
Q

What are intraepithelial non-acantholytic lesions?

A

Herpes Simplex
Herpes Zoster
Coxsackie Type A infections

41
Q

Which group of people does erythema multiforme affect most?

A

Relatively common in mild form
Predominantly young patients- 20-40 year olds, male more common
Can be recurrent

42
Q

What is the aetiology of erythema multiforme?

A

50% unknown aetiology
Some related to HSV and mycoplasma infection
Drug related- sulphonamides, contraceptive pill, penicillin
May be immune complex mediated as IgM and C3 often found in blood vessels and basement membrane of early lesions

43
Q

What is the clinical presentation of erythema multiforme?

A

Symmetrical, 1-2cm erythematous macules, papules, blisters, ulceration.
On elbows, knees, extensor surfaces of extremities
Target iris or bull’s eye lesions
Itching or burning sensation
45% have mucous membrane involvement- mouth and lips

44
Q

What is the histology of erythema multiforme?

A

Inter and intracellular oedema
Keratinocyte necrosis leading to subepithelial and intraepithelial vesicles
Vesicle formation in epithelium and sub-epithelium
Inflammatory infiltration

45
Q

What are the symptoms of Stevens-Johnsons syndrome?

A

Painful haemorrhagic oral lesions
Erosive genital lesions
Severe conjuctivitis
Fatal in 5% of cases

46
Q

What is the definition of pemphigoid group?

A

A general term applied to several immune-mediated blistering diseases producing autoantibodies to the hemidesmosomes and the epithelial basement membrane.
Results in subepithelial vesicle formation

47
Q

What are the clinical subtypes of pemphigoid related to?

A

Which antibodies are produced against different basement membrane/desmosome components.

48
Q

What are the 2 types of pemphigoid?

A

Bullous pemphigoid- mainly skin
Muocus membrane pemphigoid- mainly mucosa
Oral manifestations occur in almost all MMP patients, and are very rare in BP patients.

49
Q

What do desmosomes and hemidesmosomes use to attach?

A

Desmosomes use cadherins to attach to other cells
Hemidesmosomes use integrins to attach to the basement membrane.

50
Q

What is the lamina lucida/lamina densa?

A

Component of the basement membrane found between the epithelium and underlying connective tissue.

51
Q

What proteins do the plaque contain?

A

BP230 and BP180
Mediates attachment of keratin to the hemidesmosomal plaque

52
Q

What attaches the lamina lucida and lamina densa?

A

Laminin

53
Q

What are found between the laminin molecules?

A

Collagen 7- attaches basement membrane to connective tissue

54
Q

Which people does mucous membrane pemphigoid/ciatricial pemphigoid affect most?

A

Mainly in women- over 50 years old

55
Q

What is the clinical presentation of MMP and cicatricial pemphigoid?

A

Mouth almost always affected, sometimes only area- oral pemphigoid
Ulcerations heal slowly leaving scarring- cicatricial pemphigid
90% seen in gingiva- desquamative gingivitis

56
Q

What are the bulla like in MMP/cicatricial pemphigoid?

A

Tense- unlike pemphigus as whole epithelium is inact
Occasionally haemorrhagic

57
Q

Where else are bulla found in MMP?

A

Ocular, nasal, larynx, pharynx, oesophagus, genital mucosa

58
Q

What is the histology of MMP?

A

Separation of epithelium from the lamina propria- bulla with thick roof
As vesicles develop you get infiltration by neutrophils, eosinophils, perivascular infiltration- mainly lymphocytes

59
Q

What is the immunofluorescence results of mucous membrane pemphigoid?

A

Linear binding of IgG, IgA, C3 in the basement membrane zone
Antibodies against BP180
Circulating antibodies in serum detected in 80% of patient

60
Q

What is dermatitis herpetiformis?

A

Autoimmune blistering of skin
Has variable oral presentation in 75%
Mild form erythema, severe forms will get extensive ulceration

61
Q

What is the immunofluorescence of dermatitis herpetiformis?

A

Granular IgA deposits in connective papilla

62
Q

What are the antibodies against in dermatitis herpetiformis?

A

Gliadin
Endomysium, reticulin
Epidermal transglutaminase 3

63
Q

What are the vesicles like in dermatitis herpetiforma?

A

Subepithelial vesicles with neutrophils then later eosinophils

64
Q

What do the patients have in dermatitis herpetiforma?

A

90% have gluten hypersensitivity
Jejunal villous atrophy
Increased infiltration of intraepithelial lymphocytes

65
Q

What is linear IgA disease?

A

Rare
Shares similarities with dermatitis herpetiformis and bullous pemphigoid
Subepithelial bullae with neutrophils

66
Q

What is the immunofluorescence of linear IgA disease?

A

Linear IgA deposits along the basement membrane
Only some patients may have gluten hypersensitivity

67
Q

What is epidermolysis bullosa group?

A

Made of 30 diseases, subepithelial bullae in response to minimal trauma

68
Q

What is the cause of epidermolysis bullosa?

A

Abnormal keratins or basement membrane constituents

69
Q

What is EB simplex epidermolysis bullosa?

A

Most common, autosomal dominant, intraepidermal
Weber-Cockayne affects palms/soles, non-scarring
Kallin syndrome- autosomal recessive, alopecia, anodontia or hypodontia
Generalised types- 7

70
Q

What is EB junctional epidermolysis bullosa?

A

6 types- intra lamina lucida

71
Q

What is EB dystrophic?

A

Sub-lamina lucida, 8 types

72
Q

What is EB acquisita?

A

Associated with tissue bound and circulating autoantibodies specific to type 7 collagen

73
Q

What is angina bullosa haemorrhagica?

A

Subepithelial haemorrhagic bullae- 1-3cm in size, in middle aged/elderly patients
Usually solitary- unrelated to thrombocytopaenia

74
Q

Where is angina bullosa haemorrhagica lesions found?

A

Any place of oral mucosa, commonly soft palate

75
Q

What is angina bullosa haemorrhagic associated with?

A

Minor trauma
Hot foods
Steroid inhalers
Dental/periodontal treatment

76
Q

What is the histology of angina bullosa haemorrhagica?

A

Mild, non-specific mononuclear inflammatory cell infiltrate generally limited to the lamina propria

Oral candidiasis (18 decks)

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