A9. Autoimmune bullous skin diseases
Bullous pemphigoid
- In elderly
- Benign disease, responds well to treatment
- Most common autoimmune bullous disease
- Pathomechanism
- Autoantibodies against hemidesmosomes
- Clinical features
- Prodromal stage
- Weeks before onset of blistering
- Urticarial lesions
- Large, tense, subepidermal bulla
- Intensely pruritic
- Lower limbs, axilla
- Rarely oral involvement
- Diagnosis
- Negative Nikolsky
- Histology and immunofluorescence
- Treatment
- Prednisone (0,5 – 1 mg/bwkg)
- Azathioprine, immunosuppressants
Pemphigus vulgaris
- In middle-aged, elderly
- Deadly without treatment
- Pathomechanism
- Autoantibodies against desmosomes
- Acantholysis – loss of cell-cell adhesion
- Clinical features
- Painful, flaccid, intraepidermal (superficial) blisters
- -> lesions rupture
- -> erosions and crusts
- -> re-epithelialization with hyperpigmentation but no scarring
- No pruritus
- Lesions often begin in oral mucosa then to body parts exposed to pressure
- Diagnosis
- Positive Nikolsky – Skin comes off with scraping
- Histology and immunofluorescence
- Treatment
- High-dose prednisone (1-2 mg/bwkg)
- Azathioprine, other immunosuppressants
Dermatitis herpetiformis
- In young adults
- Risk factors
- Coeliac disease
- Iodide sensitivity
- HLA-DR3
- HLA-DQ2
- Pathomechanism
- Tissue transglutaminase and epidermal transglutaminase antibodies
- Clinical features
- Chronic, recurrent
- Tense, grouped, subepidermal vesicles
- Herpetiform appearance, hence the name
- Intensely pruritic
- Bilateral, symmetrical distribution
- No mucosal involvement
- Diagnosis
- Coeliac antibodies
- Histology and immunofluorescence
- Treatment
Epidermolysis bullosa
- Group of disorders where subepidermal blisters form following trauma
- Not autoimmune
- Epidermolysis bullosa simplex
- Junctional epidermolysis bullosa
- Dystrophic epidermolysis bullosa
- Epidermolysis bullosa acquisita