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20759934
Bullous diseases
Descripción
First Year Dentistry Mapa Mental sobre Bullous diseases, creado por sanna pathy el 07/02/2020.
Sin etiquetas
oral diseases
dentistry
first year
Mapa Mental por
sanna pathy
, actualizado hace más de 1 año
Más
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Creado por
Zainab Patel
hace más de 4 años
Copiado por
sanna pathy
hace más de 4 años
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Resumen del Recurso
Bullous diseases
Pemphigus
Paraneoplastic pemphigus
Variants
Lichenoid like
Pemphigus-like
Pemphigoid-like
Erythema-multiforme-like
= muco-cutaneous autoimmune disorder assoc. with neoplasia
Most common = lymphocytic malignancies
Non hodgkins lymphoma
Lymphocytic leukemia
Castleman's disease
Severe muco-cutaneous lesions with >1 disease pattern
Bilateral, widespread
unresponsive to treatment
Can cause RS failure
Pemphigus folaceus
No mucosal involvement
Pemphigus vulgaris
Treatment
Mild
Systemic steroids
No response -> + Mycophenolate mofetil
Moderate
Systemic steroids + Mycophenolate mofetil
No response -> Cyclophosphamide
Severe
Systemic steroids + Cyclophosphamide
Poor response/severe side effects
Rituximab
Photophoresis
IV antigens
Who gets it?
Men = women
20-50yrs
Jewish
Mediterranean
Death rate
If treated with steroids
10% die
If untreated
75% die
Clinical features
Nikosky's sign positive
1) Oral
Buccal mucosa, Palate, LIps
Blisters --> red erosions/ulcers
Extra-oral
2) Skin
Flaccid blisters
Painful erosions
Ulceration
Conjunctiva
Nose
Pharynx & larynx
Genitals
Diagnosis
Biopsy uninvolved site
Because high level of circulating Abs
Histology
Intra-epithelial blister
Auto-Abs against keratinocytes
Target antigen
DSG 1, DSG 3 (desmoglein)
Causes acantholysis
Direct immunofluorescence
Intercellular IgG or C3 in a net like pattern
Pemphigoid
Mucous Membrane Pemphigoid
Diagnosis
Biopsy adjacent to lesion
Direct immunofluorescence
IgG, IgA & C3 at basement membrane
Histology
Subepithelial blister
Separation of basement membrane
Auto-abs against hemidesmosomes in BMZ
Clinical features
1) Oral
90% have oral involvement
Buccal mucosa, Palate, Gingiva, Tongue
NOT lips
Blisters -> erosions, red patches
Desquamative gingivitis
Nikolsky sign positive
2) Eye
Sight-threatening
Conjunctivitis, symblepharon
3) Genitals, nose, pharynx, larynx
Elderly (50-60s) women
Treatment
1st line
Topical steroids
Clobetasol propionate in orobase
For gingiva -> give splint to put steroid in
+ Anti-fungal
2nd line
1.Dapsone
Side effect = anaemia
2. Mycophenolate Mofetil
Side effect = low WBC -> increased infection risk
3rd line
Systemic steroids +/- Azathioprine
+ alendronic acid & calcium tablets to reduce osteoporosis risk
Limit steroids to 5yrs
Azathioprine side effects = vomiting, no alcohol
More common
Angina bullosa haemorrhagica
Bullous pemphigoid
Rarely oral involvement
Diagnosing bullous disorders
Check these things in a patient with blistering
Nikolskys sign
Ocular lesion
Skin lesion
Ask if lesions anywhere else e.g. genital
Tests available
Histology
1. Direct immunofluorescence
2. Indirect immunofluorescence
Salt split skin
Better than normal IFF because more sensitive to Auto-Abs
3. ELISA
Dermatitis Herpetiformis
Diagnosis
Direct immunofluorescence
IgA deposits
Men=women
Sub-epithelial blister
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