Definition :

An autoimmune vesiculobullous blistering disease characterized by intra-epithelial blister formation.

Aetiopathogenesis :

) type target IgG(of autoantibodiesCirculating 3 Desmogleincomplexes, desmosomalthe

.1and

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 .