Vesiculobullous Disease 1 Flashcards
Immune mediated disease
Hypersensitivity
- type 1
- type 2
- type 3
- type 4
- type 5
Immunogenic
- cell mediated immunity
- antibody mediated immunity
2 Types of Immunological Oral Disease
Local disease
- Aphthous ulcers
- Lichen planus
- Orofacial granulomatosis
Systemic with local effects on oral mucosa
- Erythema multiforme
- Pemphigus
- Pemphigoid
- Lupus erythematosis
- Systemic sclerosis
- Sjogren syndrome
What type is Erythema multiforme?
Type 3 hypersensitivity
- antigen antibody complexes
What is in cell mediated immunity?
- aphthous ulcers
- lichen planus
- orofacial granulomatosis
What is in antibody mediated immunity?
- pemphigus
- pemphigoid
Immunological skin disease
- linked with immunological oral disease
- share many common antigens and epitopes
- embrologically oral mucosa develops from the same precursor tissue of skin
- many blistering skin conditions can also affect mouth
Difference Epitopes vs Antigen
- antigen are big immunogenic site within a protein
- antibody will only bind to one small part, such as different epitopes in sequence of protein antigen
- antibody may only bind to one particular site of antigen
- important as antigen-antibody binding will affect shape or confirmation of protein antigen and change in shape which occurs will well detect seen clinically
- Epidermolysis Bullosa, where different epitopes invovled will determine whether disease ranges from mild immunobullous condition to a more lethal form
What happens in Immunological skin disease?
- auto-antibody attack on skin components -> cause loss of cell- cell adhesion
- split forms in skin
1. fills with inflammatory exudate
2. forms vesicles/ blister
Vesicles vs Blisters
- size of lesion involved
- blisters are larger
Desmosome/ Hemidesmosome Attachment
- involved them attach the epithelial cells to each toher and basement membrane and to protein desmoglein.
- desmoglein are the target for many antibodies involved in immunobullous diseases
- causes loss of adhesion between desmosomes allowing cell layers to split
- test to biopsy sites and look of presence of antibodies within skin tissues
How does Direct immunofluorescence work?
- if suspect there is an antibody bound to the tissue
- pemphigoid in this case, antibody (blue) bind to tissue and causing disease
- manufacturing another antibody, which has a fluorescein marker tag onto it
- second antibody will bind to first antibody already attached to the tissue
- when tissue is examined with fluorescence lighting, the fluorescein will show where the antibody is bind to the tissue
Types of Immunofluorescence?
- Direct Immunofluorescence
- Indirect Immunofluorescence- circulating antibodies not yet bound to tissues
About direct immunofluorescence?
- gold standard
- looks for antibody bounds to tissue
- useful test when its is immunobullous condition
- remember a sample biopsy for immunofluorescence, must not be put into a formaline containing transport medium
- will cause binding sites to be lost
- must be transport fresh and process quickly
About indirect immunofluorescence
- looks for CIRCULATING antibody not yet bound to tissues
- detected by immunofluorescence from a plasma sample
- less reliable as a diagnostic aid
- good for monitoring disease like disease activity, looking for levels of antibody in disease such as Pemphigus can give a good guide for tx need
Which test to do for a new pt?
DIF is always preferred than IIF
Examples of Vesiculobullous conditions
- Erythema Multiforme
- Pemphigus
- Pemphigoid
- Angina Bullosa haemorrhagica
- Bullous Lichen Planus (lichen pemphigoides)
What is erythema multiforme?
- ## spectrum disorder caused by immunogenic related skin and mucosa ulceration
How is erythema multiforme formed?
- in the body, an antigen presents, which is targeted by an antibody
- there is usually an antigen which has been met before and therefore a prompt immune response results quickly generating antibodies from memory B cells
- these antigens and antibodies combine within circulations and this large complex is unable to pass through capillaries
- when complex gets to the tissues, it becomes wedges and activates compliment within blood vessels
- causing a perivascular inflammatory response
- pt will get blistering or ulceration of tissue
- depends upon individual antigen-antibody complex
- hence, some may get skin lesions and no genital lesions
- spectrum disorder
- most extreme form is known as Stevens Johnson Syndrome
What do you call a severe multisystem involvement fr Erythema Multiforme?
Stevens- Johnson syndrome
- skin, conjunctivae, nose, pharynx, mouth genital
About erythema multiforme
- acute onset
- M>F
- skin as a target lesion
- mucosa- ulcers
- young males- recurrent within a short period of time (late teens, ulceration lasting 2-3 weeks)
Aetiology
- drugs
- herpes simplex-> leads to type 3 sensitivity
- mycoplasma
Lips and anterior part of mouth
- crops, heal in 2 weeks
- very painful- unable to eat/ drink (dehydration)
Management of Erythema Multiforme
For oral lesions
1. Urgent medical therapy
- systemic steroids (up to 60mg/day)- Prednisolone
- systemic Aciclovir
- Encourage fluid intake
- may need IV fluid if unable to drink - Encourage analgesia
- If recurrent problems - consider prophylactic aciclovir daily (400mg/twice daily)
- allergy test for environmental triggers
- infective agent -> Mycoplasma- fungal infection
Angina Bullosa Haemorrhagica
- commonest oral blistering condition
- blood filled blister in oral mucosa
- usually painless
- BM and soft palate are normally common sites
- rapid onset-> appear in a few minutes, last about an hour then burst (mildly symptomatic for a day or so)
- initiated by minor trauma, ie: eating
- heal with no scarring within days
- no aetiology
Management of ABH
- non- specific ulceration
- DIF -ve
- IIF -ve
- no platelet/ coagulation defect
- may recur
Management for symptomatic
- chlorhexidine MW
- Difflam spray until lesion heals
- normally no tx available
- reassure pt that is benign
- explain triggers and course of disease, ie: eating, use of steroid inhalers
- avoid using inhalers
- will not spread into systemic issues
Resolving Angina Bullosa Haemorrhagica
- left with a small ulcer
- remember to ask pt if they start as blisters or ulcers
