PEMPHIGUS VULGARIS

BY DR KANWAL FATIMA House officer at ORAL DIAGNOSIS

department ISRA DENTAL COLLEGE

DEFINITION

it is an autoimmune, inta-epithelial, blistering disease affecting the skin and mucous membrane.

Skin blisters Mucous membrane blisters

CLINICAL FEATURES

• Bullous eruption or patches of erythema.• Bullae on skin, occasionally blood filled.• Multiple bullae rupture to leave tissue tags

and painful eruptions.• Intact bullae in mouth rare due to fragility.• Mouth shows patchy erosions.• Oral symptoms before cutaneous lesions.

• Blisters rupture and leave painful ulcers.• Nickolsky’s sign is positive.• Confluence of bullae increases size and

discomfort

PATHOGENESIS

• Intra-epithelial formation • Loss of intra-cellular adhesion (acantholysis)• Ig-G + Ig-G 4 atuo antibodies.• Intra-cellular proteolytic enzyme activated.• Complement system plays role as well.• Severity propotional to titer antibody.• Considered as a type 2 hypersentivity.

HISTOLOGICAL

IMMUNOLOGY• Immunoflorenscene at periphery of prickel

cells. (para-basal and sub surface area)• Positive on direct and indirect immuno-

florenscence.• All three layers are affected except basal layer

but this disease will occur only in prickle cell layer.

DIRECT• patient specimen•Ig G antibodies .•Auto antibodies already attached.•Autoantibody attached to patient’s tissue.

INDIRECT•Control specimen(skin or mucosa)•No IgG on tissue + patient serum.•Autoantibody in patient’s serum.

MANAGEMENT • Corticosteroids--- high doses --- 40mg• Prednisone--- (hydrocortisone) 5mg tablet

daily or alternate days.• Start with high doses later off the doses after

relief.• Maintain minimun dose in disease free state.• Immunosuppressive drugs i:e Azathioprine

(imuran), Cyclosporin (cyclophosphamide).