This document discusses the skin condition known as pemphigus, which is a serious autoimmune bullous disease affecting the skin and mucous membranes. There are several classifications of pemphigus, including pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris causes blisters in the mouth and can spread elsewhere on the body. Pemphigus is diagnosed through biopsy, microscopy, and antibody testing. Treatment involves high-dose glucocorticoids along with immunosuppressive drugs to control the autoimmune response.

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