Bullous diseases

Bullous diseases

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

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	Created by Zainab Patel about 4 years ago

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