Vesiculobullous disorders Flashcards
Vesiculobullous disorders
Mucocutaneous disorders
blister formation, vesicles, ulcers, erosions
Vesiculobullous lesions
Bulla (visual accumulation of fluid within the epi >0.5cm)
Vesicle (<0.5)
Ulcer (loss of epi, often with underlying tissue loss)
Erosion (loss of epi, follows a blister, heals without scarring)
Infectious agents
Herpes simplex
Varicella Zoster infection
Hand, foot and mouth disease
Herpangina
Measles
Immune mediated conditions
Erythema Multiforme
Pemphigus vulgaris
MMP
Dermatitis Herpetiformis
Linear IgA disease
Genetic conditions
Epidermolysis bullosa
Sub-epithelial bullous disorders
Pathology at level of attachment of epithelium and basement membrane
Erythema Multiforme
MMP
Dermatitis Herpetiformis
Linear IgA disease
Bullous lichen planus
Angina bullosa haemorrhagica
Epidermolysis bullosa
Intraepithelial bullous disorders
Pathology due to connections between epithelial cells
Pemphigus
Herpes Virus Lesions
Coxsackie virus
Erythema Multiforme
Mucocutaneous disorder
Young adults
M>F
Acute onset
Self limiting
EM Aetiology
Genetic
Microorganisms
Drugs
Chemicals
Immune factors
EM Clinical Presentation
Generally unwell
Lymphadenopathy
Widespread painful oral ulceration
Serosanguinous exudate on lips
Skin involvement
Ocular involvement
Genital involvement
EM Diagnosis
Clinical
Serology
Biopsy
EM Diagnosis
Opthalmic opinion
Self limiting
Supportive care
Antiseptic MW
Antimicrobials
Recurrent cases prophylaxis
Angina bullosa haemorrhagica
Blood filled blisters
Prodromal symptoms
prevalence uncommon
F>M
older age
ABH Aetiology
Unclear
Trauma
Corticosteroid inhalers
ABH Clinical Presentation
Rapid onset of blood filled blister formation
Non-keratinised mucosa
Break down to form large ulcers which are usually painful
ABH Diagnosis
History
Clinical Presentation
Bloods
ABH Management
Reassurance
Airway
Analgesics
Mucous Membrane Pemphigoid
Immune mediated sub-epithelial bullous disease
Autoantibodies are directed against epithelial basement membrane zone proteins (antigens)
Immune deposits result in split at basement membrane (sub epithelial split)
Affects mucous membranes +/- skin
MMP Aetiology
> 50 yrs
F>M
MMP CP
I/O
Mucosal pain
Tense bullae or vesicles which rupture to produce irregular erosions
Scarring
Desquamative gingivitis
E/O
Nasal involvement (erosions)
Genital involvement (painful erosions)
Skin involvement (tense blisters)
MMP Ocular involvement
Conjunctiva
Entropium with trichiasis
Symplepharon
Blindness
MMP Diagnosis
Clinical Presentations
Biopsy
Nikolsky positive
Immunofluorescence
Immunofluorescence
Relies on the use of antibodies to label a specific target antigen with fluorescent dye.
Allows visualisation of the target distribution in the sample under a fluorescent microscope.
What is the difference between direct and indirect immunofluorescence?
Direct: perilesional tissue/antibody binds to target antigen/less sensitive test than indirect
Indirect: serum sample/two stage process/more sensitive
Management of MMP
Local: meticulous oral hygiene/SLS free tp/Analgesic mouth rinses/topical corticosteroid preparations
Systemic: prednisolone/azathioprine/mycophenolate mofetil
Pemphigus Vulgaris
Potentially life threatening chronic autoimmune disease. Characterised by epithelial blistering of mucocutaneous surfaces. Antibodies are directed against desmosomes causing acantholysis. Immune deposits result in an intrapeithelial split.
PV Epidemiology
0.5-3.2 cases per/100 000
40-60 year olds
M:F
PV Aetiology
Drugs
Systemic conditions
PV CP
Fragile bulla
Large painful irregular erosions
Desquamative gingivitis
Nikolsky sign
PV Diagnosis
Clinical presentation
Confirmed on biopsy
PV Histology
Intraepithelial separation (acantholysis)
Tomb stone appearance
Management of PV
Local: good oral hygiene/SLS free tp/antiseptic mouth rinse/topical steroid preparations
Systemic: corticosteroids/azathioprine/rituximab/mycophenolate mofetil
