Pemphigus is a kind of skin Bullous Disease

1. PEMPHIGUS
V.Prashanth
Faculty of Medicine
University of Jaffna

2. BULLOUS DISEASE
• BLISTERS
• FILLED WITH FLUID
• SKIN OR MUCOUS MEMBRANE
• E.g.:-
– CHICKENPOX
– HERPES
– IMPETIGO
– POMPHOLYX
– ECZEMA
– PEMPHIGUS
– PEMPHIGOID
– INSECT BITE REACTION

3. PEMPHIGUS
• SERIOUS
• ACUTE OR CHRONIC
• BULLOUS
• AUTOIMMUNE DISEASE
• SKIN AND MUCOUS MEMBRANES
• ACANTHOLYSIS.

4. CLASSIFICATION
• PEMPHIGUS VULGARIS
– GENERALIZED
– LOCALISED
– DRUG INDUCED
• PEMPHIGUS FOLIACEUS
– GENERALIZED
– LOCALIZED
– FOGO SELVAGEM: ENDEMIC
– DRUG INDUCED
• PARANEOPLASTIC PEMPHIGUS
• IgA PEMPHIGUS

5. EPIDEMIOLOGY
• PV
– RARE
– JEWS, MEDITERRANEAN, INDIAN
SUBCONTINENTS
• PF
– RARE
– ENDEMIC:- FOGO SELVAGEM
• AGE:- 40-60YRS
• SEX:- MALE=FEMALE

6. ETIOLOGY & PATHOGENESIS
• AUTOIMMUNE
• ACANTHOLYSIS
– IgG AB AGAINST DESMOGELIN
– PV:- DESMOGELIN 3 & 1
– PF:-DESMOGELIN 1
• INTERFERE CALCIUM-SENSITIVE
ADHESION FUNCTION

7. CLINICAL MANIFESTATION
PV
• START IN ORAL
MUCOSA
• INITIALLY
LOCALLISED
• PAINFUL & TENDER
• ROUND OVAL
VESSICLE& BULLAE
WITH SEROUS
CONTENT
• FLACID, EASILY
RUPTURED

8. CLINICAL MANIFESTATION
• LOCALIZED OR
GENERALIZED
• NICOLSKY SIGN
• SITES:- SCALP FACE
CHEST AXILLA
• MUCOUS
MEMBRANE:-
– BULLAE RARELY

9. CLINICAL MANIFESTATION
PF
• SEBORRHEIC AREA
• SCALY, CRUSTED-
ERYTHEMATOUS
PVeg
• INTERTRIGINOUS REGION,
PERIORAL AREA, NECK
AND SCALP
DRUG INDUCED PV
• CAPTOPRIL,
• D-PENICILLAMINE
Pemphigus foliaceus

10. CLINICAL MANIFESTATION
PNP
• MUCOUS MEMBRANE
PRIMARILY
• COMBINE FEATURES
OF PEMPHIGUS
VULGARIS &
ERYTHEMA
MULTIFORME Paraneoplastic pemphigus

11. DIAGNOSIS
• DIFFICULT
– FROM ALL FORM OF ACQUIRED BULLUS
DISEASE
• BIOPSY
• MICROSCOPY
• AB DIRECTION IN SERUM
• DIRECT IF
• ELISA

12. MANAGEMENT
• GLUCOCORTICOIDS
– PREDNISOLONE (60-100mg)
• CONCOMITANT IMMUNOSUPPRESSIVE
THERRAPY
– AZATHIOPRINE (2-3mg/kg)
– MYCOPHENOLATE MOFETIL(1g tds)
– IV-Ig (2g/kg every 3-4weeks)
– RITUXIMAB

13. MANAGEMENT
• OTHER MEASURES
– CLEANSING BATH
– WET DRESSING
– ANTIMICROBIAL
– FLUID & ELECTOLYTE CORRECTION
• MONITORING
– (10% of patients may die due to complication of
disease or side effect of Rx)

14. REFERNCES…..
• Kumar and Clarks Clinical Medicine (8th
Edition)
• ABC of Dermatology (4th Edition)
• Fitzpatrick’s colour atlas and synopsis of
clinical dermatology (6th Edition)
THANK YOU