Vesiculobullous disease Flashcards
Name 3 immunological oral diseases that are local to the oral cavity
Aphthous ulcers
Lichen planus
Orofacial granulomatosis
Name 6 systemic diseases that have local effects on the oral mucosa (immunological oral diseases)
Erythema Multiforme
Pemphigus
Pemphigoid
Lupus erythematosis
Systemic Sclerosis
Sjogren’s Syndrome
What type of immunity are aphthous ulcers, lichen planus and OFG?
Cell-mediated immunity
How are blisters formed in immunological skin diseases?
Auto-antibody attack on skin components causing loss of cell-cell adhesion
Split forms in skin - fills with inflammatory exudate and forces layers of skin apart = blister is formed.
What technique is used for diagnosing vesiculobullous condition?
Direct immunofluorescence
What technique is used for monitoring vesiculobullous diseases?
Indirect immunofluorescence
Not always useful for diagnosis
What is erythema multiforme?
A spectrum auto-immune disease considered to be a reaction to an outside stimulus such as infection or medication.
What type of hypersensitivity reaction is erythema multiforme?
Type 3 - reaction mediated by immune complexes
What is the urgent medical treatment for oral lesions of erythema multiforme?
Systemic steroids - up to 60mg/day (high dose)
Systemic aciclovir - secondary to need for steroids
What should you prescribe for recurrent problems of erythema multiforme?
Prophylactic aciclovir - 400mg twice daily
What is general management for erythema multiforme?
Encourage fluid intake
Encourage analgesia
Allergy test - wide variety of environmental triggers
What is angina bullosa haemorrhagica?
Blood-filled tight blisters on the oral mucosa - most common oral blistering condition
Buccal mucosa and soft palate are most common sites
Rapid onset
Last about 1 hour then burst
What is management for angina bullosa haemorrhagica?
Symptomatic relief rather than exact treatment - CHX mouthwash or benzydamine (difflam) spray.
To diagnose WHICH condition would you want to take a PERI-LESIONAL BIOPSY?
Pemphigoid
If you take biopsy of blister - often no epithelium will be found.
What is the most common vesiculobullous condition in primary care?
Pemphigoid
What are the clinical differences between pemphigus vulgaris and mucous membrane pemphigoid?
Pemphigus affects the outer layer of the skin (epidermis) and causes lesions and blisters that easily rupture- mucosal ulceration/ erosion.
Pemphigoid affects a lower layer of the skin, between the epidermis and the dermis, creating tense blisters that do not break easily.
What would typically be seen in direct immunofluorescence for someone with pemphigoid?
Linear staining along the basement membrane
C3 and IgG detected in this area in ‘standard’ pemphigoid
What would typically be seen in direct immunofluorescence for someone with pemphigus?
Basket-weave pattern surrounding each of the epithelial cells.
What are key characteristics of pemphigus?
RARELY see intact bullae - eroded mucosa and loss of epithelial covering. Surface easily lost.
Typically oral lesions occur before skin.
What are the commonest antibodies detected in pemphigus and pemphigoid?
C3 and IgG
What are tzank cells and where are they characteristically seen in?
Broken off epithelial cells due to weakened attachment - seen in histopathology for pemphigus
What does the ELISA test do in pemphigus vulgaris?
Detects antibodies against desmoglein 1 and desmoglein 3
What virus is erythema multiforme closely associated with?
Herpes simplex virus
What type of hypersensitivity reaction are pemphigus vulgaris and mucous membrane pemphigoid?
Auto-immune type II hypersensitivity reaction
What are the histological difference between pemphigus and pemphigoid?
Pemphigus - SUPRA-BASAL split. Autoantibodies attack DESMOSOMES. Presence of Tzank cells
Pemphigoid - SUB-EPITHELIAL split, autoantibodies attach HEMIDESMOSOMES
What is the topical management for pemphigus and pemphigoid?
betamethasone mouthwash (0.5mg 2-3x/ day
Beclomethasone inhaler 50mg 2-3x/ day
What is the systemic management of pemphigus and pemphigoid?
Systemic steroids
Immunomodulators - azathioprine, mycophenolate
What are the different types of pemphigoid?
Bullous - skin
Mucous membrane pemphigoid - ALL - eye, genital, oral
Cicatricial pemphigoid - mucosal with SCARRING
What is the difference between DIRECT immunofluorescence and INDIRECT immunofluorescence?
DIRECT - most useful test of vesiculobullous condition. Antibody bound tissue targeted in DIF
INDIRECT - circulating antibody not yet bound to tissue
Not always useful for diagnosis - often good for monitoring disease activity
