PemphigusVulgaris

Pemphigus Vulgaris By

Shaima Al-Kasem

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.