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Oral Disease - Soft tissue > Blistering disease > Flashcards

Blistering disease Flashcards

1
Q

What are the classifications of vesiculobullous disorders

A

1) - Congenital or acquired

2) Based on where the skin separates: subcorneal, intra-epidermal and subepidermal

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2
Q

What type of vesiculobullous disorder does not have oral mucous membrane involvement?

A
  • Subcorneal as it separates below the stratum corneum
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3
Q

Where does intra-epidermal blistering originate?

A

Within the keratinocyte layer

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4
Q

Where does sub-epidermal blistering originate?

A

Between the dermis and epidermis

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5
Q

Describe the pathology of intraepidermal blistering

A

There is loss of cell to cell adhesion within the epithelium and the target antigens are within the desmosomes

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6
Q

Describe the pathology of subepithelial blistering

A

There is loss of adhesion between the epithelial layer and connective tissue layer, and the target antigens are in the hemidesmosome

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7
Q

What disease is associated with intraepidermal blistering

A

Pemphigus

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8
Q

What diseases are associated with subepithelial blistering

A

All variations of pemphigoid
Linerar IgA dermatosis
Epidermolysis bullosa acquisita
Dermatitis herpetiformis

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9
Q

How does the type of structural protein affected affect the severity and scarring potential?

A

The deeper the protein is, the more severe the phenoype and the greater the risk of scarring - e.g. sublamina densa type VII collagen

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10
Q

What is the most common target antigen in cutaneous blistering disorders?

A

Bp180

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11
Q

What is epidermolysis bullosa?

A

A group of rare inherited skin disorders due to mutations in genes encoding structural components of skin, resulting in skin or mucosal fragility

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12
Q

What does the severity of epidermolysis bullosa depend on?

A
  • Recessive trait more severe than dominant
  • The type of structural protein affected
  • The specific mutation
  • Penetrance
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13
Q

What are the three types of epidermolysis bullosa

A

EB simplex
EB junctional
EB dystrophic

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14
Q

Describe features of recessive dystophic epidermolysis bullosa

A

Stocking scarring (feet), curled fingers, oral and GIT scarring, short stature, life expectancy around 30 and tend to die due to squamous cell carcinoma

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15
Q

Describe EB simplex

A

Most common, usually autosomal dominant and causes localised blisters or grouped vesicles with limited mucosal involvement

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16
Q

Describe EB junctional

A

Most severe type, autosomal recessive type, chracterised by widespread blistering, scarring, granulation tissue, severe mucosal involvement, dental pitting, alopecia and nail dystophy

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17
Q

Describe EB dystrophic

A

Can be autosomal dominant or recessive, characterised by haemorrhagic blisters, scarring, severe mucosal involvement, physical and sexual retardation, and high mortality rates

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18
Q

What are the primary acquired blistering diseases

A
  • Pemphigus
  • Pemphigoid
  • Linear IgA disease
  • Epidermolysis bullosa acquisita
  • Dermatitis herpetiformis
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19
Q

What are secondary acquired blistering diseases?

A 
Trauma e.g. blisters from tight shoes 
Infections e.g. herpes 
Acute eczema 
Drug reactions 
Metabolic diseases - diabetes
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20
Q

History taking for blistering disease

A
  • Age of onset
  • Duration of lesions
  • Any other lesions on the body
  • Precipitating events e.g. drug history
  • MH - systemic illness or coealic
  • Family history of autoimmune disease
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21
Q

How to examine a blister

A
  • Site (localised or generalised)
  • Morphology and evidence of scarring/erosion
  • Colour of background skin (normal or erythema)
  • Nikolsky sign
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22
Q

IX for blisters

A
  • Nikolsky sign
  • FBC
  • Antibody screen
  • Biopsy
  • Direct immunoflouresence
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23
Q

Management of blistering diseases

A
  • Assess need fo admission to hospital - fluid balance, thermoregulation etx
  • Topical tx for infection if present
  • Systemic therapy
  • Referral to opthal or ENT
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24
Q

What systemic therapy is used in blistering diseases

A

Prednisolone +/- steroid sparing agents

Antibiotics if required

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25
Q

List the types of intra-epithelial blisters

A

Pemphigus vulgaris/vegetans
Pemphigus foliaceous
Paraneoplastic pemphigus

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26
Q

What is pemphigus vulgaris?

A

An uncommon autoimmune disease characterised by vesicles or bullae on the skin and mucous membranes

27
Q

Epidemiology of pemphigus vulgaris

A

Female
40-60
All races (except asian and jewish)

28
Q

Describe the clinical appearance of pemphigus vulgaris

A
  • Painful lesions on the mucous membranes and/or skin (2/3 have oral lesions)
  • Fragile bullae and erosions on normal skin
  • Positive nikolsky sign
29
Q

What is a positive nikolsky sign?

A
  • Gentle lateral pressure to the mucosa results in the formation of a vesicle of bulla
30
Q

Results of direct immunoF for pemphigus vulgaris

A

Intercellular IgG and C3

31
Q

Histological features of pemphigus vulgaris

A

Acantholysis due to antibody-antigen binding (separates and degrades desmosomal attachment and epithelium falls apart)
- Clefts form superficial to basal cells and may extend to form intraepithelial vesicles

32
Q

What are the target antigens for pemphigus vulgaris

A

DSG 3 (130kd) and/or DSG1 (160kd)

33
Q

What type of demoglein is dominant in the mucous membranes?

A

DSG3 (in comparison to dsg1 which is more dominant in the skin)

34
Q

Describe the oral lesions in pemphigus vulgaris

A
  • Site: BM, tongue and palate
  • Initial erosions are superficial ulcers that are fragile (rare to see intact intraorally) but residual erosions have ragged edges and are painful/tender
  • Serum exudate
  • Desquamative gingivitis
  • Scattered vegetations or haemorrhagic lesions on the lips
35
Q

Why are intraoral bulla rare?

A

The keratin layer is not thick enough to form a strong roof for a blister

36
Q

Describe skin lesions in pemphigus vulgaris

A
  • Vesicles or bulla (mm or cms)
  • Contain clear liquid initially, may become purulent or heamorrhagic
  • Rupture results in painful erosions (can become secondarily infection)
37
Q

Presentation of paraneoplastic pemphigus

A

Periocular and perioral erosions with haemorrhagic lesions around lips and eyes +/- flexural skin involvement

38
Q

What does paraneoplastic pemphigus indicate

A

Underlying malignancy - commonly NHL or CLL

39
Q

Describe the pathology of paraneoplastic pemphigus

A

Autoantibodies cross-react with the tumour and epidermal antigens, resulting in a t cell response

40
Q

Describe the appearance of pemphigus foliaceous

A

Superficial red skin lesions, which may erode (BUT DO NOT BLISTER) on the face, neck, scalp and upper trunk
- NO INTRAORAL LESIONS !!!!!

41
Q

What are the subepidermal blisters

A
  • Bullous pemphigoid
  • Mucous membrane pemphigoid
  • Linear IgA disease
  • Epidermolysis bullosa acquisita
42
Q

Epidemiology of bullous pemphigoid

A

The most common immunobullous disease

Commonly affecting the elderly

43
Q

Describe the presentation of bullous pemphigoid

A

Pruitic (but not painful), tense blisters with erythematous background
Located on the lower limbs (localised), or generalised
Oral involvement is rare (<30% and often transient)

44
Q

What are the target proteins of bullous pemphigoid

A

BP180
BP230
NC16A

45
Q

Tx of bullous pemphigoid

A 
Topical corticosteroids 
Tetracycline 
Dapsone 
Prednisolone 
Azathioprine
46
Q

Epidemiology of mucous membrane pemphigoid

A

Most common ORAL immunobullous disease (however it is still uncommon
Female predilection
Mean age of 60

47
Q

Pathology of mucous membrane pemphigoid

A

Loss of attachment and separation of full thickness epithelium from connective tissue at the level of the basement membrane

48
Q

Presentation of mucous membrane pemphigoid

A
  • Site: mouth, eyes, nasopharynx, oesophagus, skin, genitals
  • Oral lesions - attached gingiva or palate (keratinised mucosa) or on the tongue or BM - bulla often intact
  • Bleeding of oral lesions gives the appearance of blood blisters
  • Rupture results in raw ulcers with well defined marings and small tags of torn epithelium
  • Desquamative gingivitis
  • Skin involvement is minimal or absent
49
Q

Target proteins in MMP

A

BP180
BP230
Alpha-6-b4
Laminin 5

50
Q

Tx of MMP

A
  • CHX, hydrogen peroxide of difflam mouthwash
  • Topical corticosteroid (betamethasone)
  • Systemic corticosteroids - dapsone, prednisolone, azathioprine
51
Q

What is linear IgA disease

A

Rare, autoimmune blistering disease predominantly affecting the skin, but has some mucosal involvement

52
Q

Describe oral lesions in linear IgA disease

A

Blisters, erosions and ulcers typically on the tongue

53
Q

Direct IF for linear IgA disease

A

IgA visible along the membrane

54
Q

Describe the features of epidermolysis bullosa acquisita

A
  • Lesions affecting cutaneous areas prone to friction - elbows, knees and toes
  • Scarring causes ankyloglossia, micostomia and oseophageal strictures
55
Q

Epidemiology of dermatitis herpetiformis

A
  • Dermatological complication of coealic

- 20-40 yo

56
Q

Presentation of dermatitis herpetiformis

A

Intensely itchy rash found on extensor surfaces (elbows and buttocks)
RARELY affects oral mucosa

57
Q

Pathology of dermatitis herpetiformis

A

Autoantibodies directed against skin and mucosal transglutaminase enzymes on the keratinocyte surface

58
Q

Ix of dermatitis herpetiformis

A

Folate
Jejunal biopsy
Transglutaminase and anti-endomysial antibodies

59
Q

Tx of dermatitis herpetiformis

A

Dapsone

Gluten free diet

60
Q

Differential diagnosis of blistering diseases

A
  • Erythema multiforme
  • SJS/TENS
  • Bullous lichen planus
  • Angina bullosa
61
Q

What is erythema multiforme?

A

Hypersensitivity reaction usually triggered by infections (HSV, mycoplasma, syphillis) or drugs (penicillin or phenytoin) resulting in self-limiting, episodic lesions on the skin and mucous membranes

62
Q

Describe the presentation of erythema multiforme

A
  • Target like lesions (centre is dusky or purpura with outer erythemaous ring - these may ulcerate) on the skin
  • Ulcers on the lips and tongue
  • Mild fever
63
Q

Tx of erythema multiforme

A

Corticosteroids (symptomatic relief)

Tx of underlying infection if present

Oral Disease - Soft tissue (12 decks)

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