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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
List the types of intra-epithelial blisters
Pemphigus vulgaris/vegetans Pemphigus foliaceous Paraneoplastic pemphigus
26
What is pemphigus vulgaris?
An uncommon autoimmune disease characterised by vesicles or bullae on the skin and mucous membranes
27
Epidemiology of pemphigus vulgaris
Female 40-60 All races (except asian and jewish)
28
Describe the clinical appearance of pemphigus vulgaris
- 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
What is a positive nikolsky sign?
- Gentle lateral pressure to the mucosa results in the formation of a vesicle of bulla
30
Results of direct immunoF for pemphigus vulgaris
Intercellular IgG and C3
31
Histological features of pemphigus vulgaris
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
What are the target antigens for pemphigus vulgaris
DSG 3 (130kd) and/or DSG1 (160kd)
33
What type of demoglein is dominant in the mucous membranes?
DSG3 (in comparison to dsg1 which is more dominant in the skin)
34
Describe the oral lesions in pemphigus vulgaris
- 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
Why are intraoral bulla rare?
The keratin layer is not thick enough to form a strong roof for a blister
36
Describe skin lesions in pemphigus vulgaris
- 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
Presentation of paraneoplastic pemphigus
Periocular and perioral erosions with haemorrhagic lesions around lips and eyes +/- flexural skin involvement
38
What does paraneoplastic pemphigus indicate
Underlying malignancy - commonly NHL or CLL
39
Describe the pathology of paraneoplastic pemphigus
Autoantibodies cross-react with the tumour and epidermal antigens, resulting in a t cell response
40
Describe the appearance of pemphigus foliaceous
Superficial red skin lesions, which may erode (BUT DO NOT BLISTER) on the face, neck, scalp and upper trunk - NO INTRAORAL LESIONS !!!!!
41
What are the subepidermal blisters
- Bullous pemphigoid - Mucous membrane pemphigoid - Linear IgA disease - Epidermolysis bullosa acquisita
42
Epidemiology of bullous pemphigoid
The most common immunobullous disease | Commonly affecting the elderly
43
Describe the presentation of bullous pemphigoid
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
What are the target proteins of bullous pemphigoid
BP180 BP230 NC16A
45
Tx of bullous pemphigoid
``` Topical corticosteroids Tetracycline Dapsone Prednisolone Azathioprine ```
46
Epidemiology of mucous membrane pemphigoid
Most common ORAL immunobullous disease (however it is still uncommon Female predilection Mean age of 60
47
Pathology of mucous membrane pemphigoid
Loss of attachment and separation of full thickness epithelium from connective tissue at the level of the basement membrane
48
Presentation of mucous membrane pemphigoid
- 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
Target proteins in MMP
BP180 BP230 Alpha-6-b4 Laminin 5
50
Tx of MMP
- CHX, hydrogen peroxide of difflam mouthwash - Topical corticosteroid (betamethasone) - Systemic corticosteroids - dapsone, prednisolone, azathioprine
51
What is linear IgA disease
Rare, autoimmune blistering disease predominantly affecting the skin, but has some mucosal involvement
52
Describe oral lesions in linear IgA disease
Blisters, erosions and ulcers typically on the tongue
53
Direct IF for linear IgA disease
IgA visible along the membrane
54
Describe the features of epidermolysis bullosa acquisita
- Lesions affecting cutaneous areas prone to friction - elbows, knees and toes - Scarring causes ankyloglossia, micostomia and oseophageal strictures
55
Epidemiology of dermatitis herpetiformis
- Dermatological complication of coealic | - 20-40 yo
56
Presentation of dermatitis herpetiformis
Intensely itchy rash found on extensor surfaces (elbows and buttocks) RARELY affects oral mucosa
57
Pathology of dermatitis herpetiformis
Autoantibodies directed against skin and mucosal transglutaminase enzymes on the keratinocyte surface
58
Ix of dermatitis herpetiformis
Folate Jejunal biopsy Transglutaminase and anti-endomysial antibodies
59
Tx of dermatitis herpetiformis
Dapsone | Gluten free diet
60
Differential diagnosis of blistering diseases
- Erythema multiforme - SJS/TENS - Bullous lichen planus - Angina bullosa
61
What is erythema multiforme?
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
Describe the presentation of erythema multiforme
- 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
Tx of erythema multiforme
Corticosteroids (symptomatic relief) | Tx of underlying infection if present

Oral Disease - Soft tissue (12 decks)

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