SPUP MEDICINE

LECTURER: Dr. MORALES

PEMPHIGUS EXIMIUS 2021
DERMATOLOGY
September, 2019

PEMPHIGUS ORAL LESION

o AUTOIMMUNE blistering disease of the skin & mucous membrane o 50 -70% of patients
o INTRAEPIDERMAL blister o Only signs 5 months before skin lesions develop
o ACANTHOLYSIS –separation of the epidermal cells from each o Painful erosions
other
o IgG directed against the cell surface of the keratinocyte

CLASSIFICATION OF PHEMPHIGUS
o Phemphigus vulgaris
 Pemphigus vegetans: Localized
 Drug induced CUTANEOUS LESION
o Flaccid blisters
o Phemphigus foliaceus o Painful erosion
 Phemphigus erythematous: Localized o (+) Nikolsky sign
 Fogo selvage: Endemic o Heal without scarring
 Drug induced

o Paraneoplastic pemphigus

o IgA pemphigus
 Sub-corneal pustular dermatosis
 Intraepidermal neutrophilic IgA dermatosis PEMPHIGUS FOLIACEUS
o Su[perficial blister with scaly crusted
Pemphigus vulgaris (PV) Pemphigus foliaceus (PF) erosion on the erythematous base
superficial pemphigus o Face, scalp, upper trunk
Clinical o Flaccid blister o Crusted scaly lesion o (+) Nikolsky sign
feature o Erosion on skin & o Flaccid bullae are difficult to find
mucous membrane o Pain and burning
Blister o Suprabasilar o Subcorneal acantholysis o Mucous membrane involvement rare
location acantholysis (Granular Layer) o Sun and heat exacerbate the disease

PEMPHIGUS VULGARIS o Pemphiugus erythematous and fogo

o M=F selvage represent localized and
o Mean age of onset : 40-60 y/o endemic variant
o More common in Jews &
people of Mediterranean
descent
o HLA-DRB1 TOPICAL THERAPY
o Potent topical or intralesional steroids
Pathogenesis o Good oral hygiene
o Pemphiugus vulgaris antigen o Potassium permanganate and topical antiseptics
 Desmoglein 1 and 3 –transmembrane glycoprotein of o Oral antifungal
desmosomes SYTEMIC THERAPY
o Prednisolone
o Pemphiugus foliaceus antigen
 1.0-1.5 mg/kg/day in combination with topical
 Desmoglein 1
 High dose Prednisolone
 120-240 mg/day for severe pemphigus
o Antibodies
o Azathioprine
 IgG against cell surface keratinocyte
 2.5 mg/kg/day
 Combination more effective
o Loss of adhesion of keratinocytes
o Oral intramuscular Gold
o Tertacylcine in combination
o Dapsone
o Cyclophosphamide
 1-3 ,g/kg/day in combination
o Cyclosporine 5 mg/kg/day
o Mycophenolate mofetil (CellCept)
 2 g/day as steroid sparing
o Plasmaphersis
 Reduces the titres of autoantibodies by 15%
o Methotrexate not effective
o High-dose IVIg
o Extracorporeal Photopheresis with PUVA
PROGNOSIS
o Refractory to all treatment
o Cause of Death
 Infection
 Sepsis
 GI bleeding
 Multiorgan failure
 Respiratory failure

TRANSCRIBERS Smile
1 of 1