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Definition :
An autoimmune vesiculobullous blistering
disease characterized by intra-epithelial blister
formation.
Aetiopathogenesis :
Circulating autoantibodies of (IgG) type target
the desmosomal complexes, Desmoglein 3
and 1 .
Epidemiology :
- Equal Incidence rate in both genders .
- Most patient presenting during the 4th and 5th
decades of life .
- Genetic and ethnic factors: Ashkenazic Jews
and Mediterranean patients .
Clinical features :
- Painful ulcers .

- Intra oral: Bullae apprear on any part of oral
mucosa and becomes ulcers, fig (1-2) .
- Extra oral : blisters appear on skin of face, back
or chest, fig(3) .
- Positive Nikolsky`s sign .
Clinical features :

Fig(1): ulcers in lower lip
Clinical features :

Fig(2): ulcers in buccal mucosa
Clinical features :

Fig(3): ulcers in skin of back
Diagnosis :
- Direct Immunofluorescence fig(4) .
- Indirect Immunofluorescence .
Diagnosis :

fig(4): Direct Immunofluorescence .
Differential Diagnosis :
- Mucous membrane pemphigoid .

- Erythema Multiforme .
- Paraneoplastic pemphigus .
- Pemphigus vegetans .
Treatment :
Based on disease severity :
* Mild :
- Topical corticosteroids .
Treatment :
* Moderate to sever :
systemic agents include :
- prednisone .
- mycophenolate mofetil .
- azathioprine .
- cyclosporine .
Pemphigus Vulgaris: Blistering Skin and Mouth Disease

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Pemphigus Vulgaris: Blistering Skin and Mouth Disease