PEMPHIGUS

V.Prashanth

Faculty of Medicine

University of Jaffna

BULLOUS DISEASE

• BLISTERS

• FILLED WITH FLUID

• SKIN OR MUCOUS MEMBRANE

• E.g.:-– CHICKENPOX

– HERPES

– IMPETIGO

– POMPHOLYX

– ECZEMA– PEMPHIGUS

– PEMPHIGOID

– INSECT BITE REACTION

PEMPHIGUS

• SERIOUS

• ACUTE OR CHRONIC

• BULLOUS

• AUTOIMMUNE DISEASE

• SKIN AND MUCOUS MEMBRANES

• ACANTHOLYSIS.

CLASSIFICATION

• PEMPHIGUS VULGARIS

– GENERALIZED

– LOCALISED

– DRUG INDUCED

• PEMPHIGUS FOLIACEUS

– GENERALIZED

– LOCALIZED

– FOGO SELVAGEM: ENDEMIC

– DRUG INDUCED

• PARANEOPLASTIC PEMPHIGUS

• IgA PEMPHIGUS

EPIDEMIOLOGY

• PV

– RARE

– JEWS, MEDITERRANEAN, INDIAN SUBCONTINENTS

• PF

– RARE

– ENDEMIC:- FOGO SELVAGEM

• AGE:- 40-60YRS

• SEX:- MALE=FEMALE

ETIOLOGY & PATHOGENESIS

• AUTOIMMUNE

• ACANTHOLYSIS

– IgG AB AGAINST DESMOGELIN

– PV:- DESMOGELIN 3 & 1

– PF:-DESMOGELIN 1

• INTERFERE CALCIUM-SENSITIVE

ADHESION FUNCTION

CLINICAL MANIFESTATIONPV

• START IN ORALMUCOSA

• INITIALLYLOCALLISED

• PAINFUL & TENDER

• ROUND OVALVESSICLE& BULLAEWITH SEROUSCONTENT

• FLACID, EASILYRUPTURED

CLINICAL MANIFESTATION

• LOCALIZED OR

GENERALIZED

• NICOLSKY SIGN

• SITES:- SCALP FACE

CHEST AXILLA

• MUCOUS

MEMBRANE:-– BULLAE RARELY

CLINICAL MANIFESTATION

PF

• SEBORRHEIC AREA

• SCALY, CRUSTED-ERYTHEMATOUS

PVeg

• INTERTRIGINOUS REGION,PERIORAL AREA, NECKAND SCALP

DRUG INDUCED PV

• CAPTOPRIL,

• D-PENICILLAMINE

Pemphigus foliaceus

CLINICAL MANIFESTATION

PNP

• MUCOUS MEMBRANE

PRIMARILY

• COMBINE FEATURES

OF PEMPHIGUS

VULGARIS &

ERYTHEMA

MULTIFORME Paraneoplastic pemphigus

DIAGNOSIS

• DIFFICULT

– FROM ALL FORM OF ACQUIRED BULLUS

DISEASE

• BIOPSY

• MICROSCOPY

• AB DIRECTION IN SERUM

• DIRECT IF

• ELISA

MANAGEMENT

• GLUCOCORTICOIDS

– PREDNISOLONE (60-100mg)

• CONCOMITANT IMMUNOSUPPRESSIVE THERRAPY

– AZATHIOPRINE (2-3mg/kg)

– MYCOPHENOLATE MOFETIL(1g tds)

– IV-Ig (2g/kg every 3-4weeks)

– RITUXIMAB

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)

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