Zusammenfassung der Ressource
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