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Vesiculobullous Conditions, Topical steroids - in vacuums formed splints…
Vesiculobullous Conditions
Terminology
Bulla
- blister >1cm that contains clear, haemorrhage or serous fluid
burst to result in erosion/ulceration
Intra-epithelial
- ulceration/separation occurs within the epithelium
Acantholtyic
- separation of keratinocytes due to loss of adhesion as a result of the failure of intra-epithelal cell junctions
Non-acantholytic
- separation of epithelial cells as a result of a viral infection
Herpes Simplex -
Primary Gingivostomatitis
Herpes zoster
coxsaxkie infections
Subepithelial
- ulceration/separation occurs within the basement membrane
as a result of production of autoantibodies against components of epithelium or basement membrane
Vesicle
- blister <1cm that contains clear, serous or haemorrhage fluid
Pemphigus
Aietology
Autoimmune - autoantibodies which target components of desmosomes in epithetlium
desmoglein 1
desmoglein 3
epidemiology
common between 30-60year olds
increased genetic component in south africans
Signs and Symptoms
oral bullae/vesicles that
rupture
very easily
oral mucosal pain which may limit function
Clincal Features
flaccid bullae/vesicles which rupture
easily
to leave areas of ulceration/erosion
Niklosky's Sign - layers of epithelium removed when rubbed
Investigations
2 types of biopsies to detect autoantibodies in patient tissue
lesion biopsy fixed in formalin, processed into wax bloxed and examined histopathologically
peri-lesional tissue (normal appearing tissue) for DIF
detects autoantibodies and they're binding site
IDIF and ELISA
patient does not want to undergo biopsy
biopsy undiagnositic
biopsy contra-indicted
Management
Referral to secondary care
recognition of signs and symptoms
Local management
Topical Steroids
Topical analgesics/mouthwash - Gelclair
Systemic Management
Prednisolone 1mg/kg
DMARD's
Rituximab
Azathioprine
Types
Vulgaris
Most common type - 70% of cases
Foliaceous
predominately affects the skin - autoantibodies target desmoglein 1
Paraneoplastic
linked to lymphoproliferative disorders e.g. lymphoma
Drug Induced
occurs <6months after commencing drug tx
ACE inhibitors - Captopril
Mucous Membrane Pemphigus
Aetilogy
Autoimmune
Autoantibodies target components of basement membrane
BP180
Laminin 332
B4 intern sub unit
Epidemology
F>M
50s with peak incidence 70s
Signs and Symptoms
Bulla/vesicles which remain
intact
oral ulceration/erosion which may limit function
Clinical Features
turgid bulla/vesicles which rupture to leave areas of deep ulceration/erosion
confined to gingiva =
desquamative gingivitis
Investigations
lesional tissue for histopathological examination
peri-lesional tissue for DIF
Management
Topical Management
Topical analgesics/ mouthwash
Goof OH for desquamative gingivitis
Systemic Managemnt
Mild
Doxycycline
Severe
Dapsone
Prednisolone
DMARDs
Topical steroids - in vacuums formed splints to apply gel to gingiva
potent
betamethasone valerate
fluocinilone
super potent
Clobetasol
