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Oral Disease for FINALS > Mucocutaneous disease > Flashcards

Mucocutaneous disease Flashcards

1
Q

What is a macule / macular lesion (3)

A

Circumscribed flat lesion
Not elevated
Not palpable

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2
Q

What is a papule / papular lesion (3)

A

Circumscribed raised lesion
Raised
Palpable

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3
Q

What is a blister? (2)

A

A fluid filled sac within or below epithelium

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4
Q

What is a vesicle + example (3)

A

A small blister <5mm diameter

e.g. Herpes simpex vesicles

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5
Q

What is a bulla + example (3)

A

A large blister >5mm diameter

E.g. pemphigus/ pemphgoid

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6
Q

Describe erosive lesions (3)

A 
Marked thinning / partial loss
of epithelium
But with a thin epithelial
covering of the connective
tissue
Usually looks red and is very
sensitive
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7
Q

Describe an ulcerative lesion (3)

A 
 Localised loss of entire
thickness of epithelium
 Exposes underlying
connective tissue
 Usually painful
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8
Q

What are the autoimmune bullous diseases and what type of hypersensitivity are they? (4)

A

Pemphigus
Pemphigoid
Dermatitis herpetiformis
-type II hypersensitivity

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9
Q

Types of mucocutaneous disease (4)

A

Autoimmune bullous disease
Epidermolysis bullosa congeita (congital anomaly)
Erythema multiforme (type III/ IV hypersensitivity)
Oral luchen planus and lichenoid reactions (type IV hypersensitivity)

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10
Q

Types of autoimmune bullous disease (3)

A

Pemphigus
Pemphigoid
Dermatitis herpetiformis

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11
Q

Features of autoimmune bullous disease (3)

A

Organ specific
Antibody-mediated (type II hypersensitivity)
Autoimmune diseases

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12
Q

Epidemiology of pemphigus (3)

A

Incidence 0.5-3.2 per 100,000
Main age group: 40-60 years
Male to female ratio = 1:1

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13
Q

What is pemphigus (1)

A

Organ specific autoimmune disease targeting skin and oral mucosa

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14
Q

Oral features of pemphigus (4)

A

Mouth involved in most cases, and is the only site involved in over half of cases
Palate, buccal mucosa and gingivae are most commonly affected
Bullae are short lived in mouth and on the skin
Large shallow non-healing ulcers are typical

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15
Q

Nikolsky’s sign (2)

A

Used in diagnosis of pemphigus

Top layers of skin slip away from lower layers when rubbed

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16
Q

Pathogenesis of pemphigus (3)

A

Circulating autoantibodies against building proteins that keep epithelial cells together
Binding protein - part of desmosomal complex, usually desmoglein3 (sometimes desmoglein 1 as well)
Autoantibody binds to desmoglein3 leading to epithelial cells separation (acantholysis) and formation of an intra-epithelial bulla

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17
Q

Cells in pemphigus (1)

A

Tzank cells

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18
Q

Investigations for pemphigus (5)

A

Biopsy of para-lesional and / or normal tissue
Send tissue to lab fresh or frozen (do not fix)
Routine histology
Direct immunofluorescence staining: used to detect whether autoantibodies are present in the patient’s tissue
Also send blood sample for indirect immunofluorescence, used to detect circulating autoantibodies

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19
Q

Immunofluorescence - pemphigus (2) **

A

Positive direct immunofluorescent staining of keratinocytes (fish net
pattern)
Autoantibodies (IgG) target Desmoglein 3 in the desmosomes that join keratinocytes

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20
Q

Sub-types of pemphigus (3)

A

Pemphigus vulgaris
Pemphigus foliaceous
Paraneoplastic pemphigus

21
Q

What is pemphigus vulgaris (1)

A

Most common (autoantibodies mainly to DG3)

22
Q

What i pemphigus foliaceous (1)

A

Lesions more superficial (autoantibodies mainly to DG1)

23
Q

What is paraneoplastic pemphigus (3)

A

Associated with a neoplasm
Usually lymphoma or chronic lymphocytic leukaemia
Extremely serious with a high morbidity and mortality

24
Q

Management of pemphigus (4)

A

Exclude cancer
Immunosuppression
Prednisolone alone or in combination with azathioprine
Occasionally other immunosuppressants or plasmaphoresis

25
Q

Intra- vs sub-epithelial bullae (5)

A

Intra-epithelial bullae (pemphigus)
-partial thickness
-very fragile
-on rupturing, basal epithelial cells remain
-therefore no stimulus to heal
-however, epithelial permeability barrier is lost, therefore infection can get in; there is loss of tissue fluids (like a burn); together these are life threatening
Sub-epithelial bullae (pemphigoid etc.)
-full thickness
-fragile
-on rupturing, exposed connective tissue
-healing by secondary intention (epithelial migration from edges, wound contraction i.e. scarring)

26
Q

Types of sub-epithelial bullous disease (3)

A 
Bullous pemphigoid (BP)
Mucous membrane pemphigoid (MMP) (Cicatricial pemphigoid)
Dermatitis herpetiformis (DH)
27
Q

Pathogenesis of pemphigoid (2)

A

Autoantibodies directed against components of the hemidesmosomes: structures gluing the epithelial cells to the basement membrane.
The targeted part of hemidesmosome varies between different types of pemphigoid.

28
Q

BP/ MMP - pemphigoid: histology (1)

A

Epithelium separates from connective tissue at the level of the basement membrane

29
Q

Features of bullous pemphigoid (3)

A

Skin usually involved with bullae and large shallow ulcers or erosions
Mouth and other mucous membranes frequently involved
Autoantibodies directed against the BP180 (BPAG1) and BP230 (BPAG2) antigens in hemidesmosomes

30
Q

Features of mucous membrane pemphigoid (6)

A

Chronic disease of the elderly
Desquamative gingivitis in >90% of cases
Buccal mucosa and palate often involved
Eyes may be severely damaged by scarring –
Cicatricial Pemphigoid
Skin lesions are rare in MMP
Autoantibodies directed against BP`230, laminin and α6β4 in hemidesmosomes

31
Q

Gingival and mucosa involvement in MMP (5)

A 
Gingival involvement in >90% cases
Other mucosal involvement:
-82.5% other oral mucosa
-48.3% conjunctiva
-8.3% skin
-7.5% nasal
32
Q

Features of MMP (5)

A

Well defined ulcers
Healing in 3-4 weeks; risk of scarring if eyes, larynx, oesophagus involved
Erythematous, friable, tender gingiva
Nikolsky sign positive
Conjunctival lesions common (up to 80%)
-symblepharon, ankyloblepharon, lid inversion

33
Q

Eye lesions in MMP/ cicatricial pemphigoid

A

Blisters and ulcers (conjunctivitis)
Trichiasis, fibrosis and scarring
Entopion plus adhesions - symblepharon

34
Q

MMP - treatment (4)

A

Steroids: topical/ systemic (if severe)
Plaque reduction: daily scrupulous home care, chlorhexidene
Tetraycycline/ nictinamide
Other immunosuppressive agents: azathioprine, cyclophosphamide, dapsone, mycophenolate

35
Q

MMP pemphoid referral (2)

A

Immediate - telephone

Needs ophthalmology opinion

36
Q

Investigations for BP/ MMP - pemphigoid (4)

A

Biopsy of para-lesional and / or normal tissue
Send tissue to lab fresh or frozen (do not fix)
Routine histology
Direct immunofluorescence staining: to detect autoantibodies in the tissue

37
Q

Immunofluorescence of BP/ MMP - pemphigoid (1)

A

Linear IgG or IgM immunofluorescence at the basement membrane
Standard Indirect immunofluorescence studies usually negative

38
Q

Management of BP/ MMP - pemphigoid (4)

A

Systemic or topical steroids are the mainstay of treatment
Sulphonamides or Dapsone may be an effective alternative to systemic steroids
Mycophenolate mofetil, an additional systemic agent
Ocular examination is essential

39
Q

Features of dermatitis herpetiformis (3)

A

Similar to BP but:
-may affect a younger age group including
children
-smaller bullae and vesicles - hence herpetiform appearance of lesions
-association with Coeliac disease (gluten enteropathy)

40
Q

Histology of dermatitis herpetiformis (1)

A

Small regions of epithelial separation at the level of the basement membrane

41
Q

Immunofluorescence: dermatitis herpetiformis (1)

A

Speckled / granular IgA immunofluorescence at the
basement membrane and in the adjacent connective
tissue

42
Q

Management of dermatitis herpetiformis (2)

A

Gluten free diet

May respond well to sulphonamides or dapsone

43
Q

Features of epidermolysis bullosa congenita (6)

A

Not autoimmune
Inherited group of conditions
Genetic defects in key proteins associated
with epithelial integrity or anchoring to the CT
The clinical picture differs depending on which
protein is defective
Mainly affects children- often present at birth
Some forms are severe, mutilating or fatal

44
Q

Features of erythema multiforme (6)

A

Acute onset, short duration ~2-3 wks
Mucocutaneous blistering disorder
Peak age range 20-30yrs
Complex pathogenesis
Some cases immune complex mediated – (Type III
hypersensitivity. Immune complexes deposited in tissues)
Some recurrent cases (Type IV hypersensitivity to herpes antigens.)

45
Q

Clinical features of erythema multiforme (4)

A 
Oral
 Haemorrhagic crusting of
lips
 Extensive irregular
mucosal ulceration
erythema and blistering
Ocular
 Conjunctivitis
Skin
 “Target” lesions
Severe Cases
 Stevens-Johnson
syndrome
46
Q

Causes of erythema multiforme (5)

A 
Single episode:
-drugs
-mycoplasma pneumonia
-radiotherapy
-idiopathic
Recurrent:
-recurrent Herpes simplex (cold sores)
47
Q

Management of erythema multiforme (7)

A 
Remove / avoid trigger
Short, reducing dose course of steroids
Chlorhexidine / Benzidamine mouthwash
Gengigel / Gelclair
Analgesics
Soft diet
May require admission for parentaral nutrition and more intensive therapy
48
Q

Management of recurrent erythema multiforme (1)

A

Prevention with systemic acyclovir

Oral Disease for FINALS (34 decks)

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