pemphigus vulgaris is an autoimmune disease. topic is covered completely in a presentation. oral medicine

1. PEMPHIGUS VULGARIS
BY DR KANWAL FATIMA
House officer at ORAL DIAGNOSIS
department ISRA DENTAL COLLEGE

2. DEFINITION
it is an autoimmune, inta-epithelial, blistering
disease affecting the skin and mucous
membrane.
Skin blisters Mucous membrane blisters

3. 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.

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

6. 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.

8. HISTOLOGICAL

10. 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.

11. 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.

12. 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).