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Pemphigus

This document summarizes information about pemphigus, an autoimmune blistering disease of the skin and mucous membranes. It is caused by IgG antibodies directed against desmoglein proteins in the skin. There are two main types: pemphigus vulgaris, which causes flaccid blisters on skin and mucous membranes, and pemphigus foliaceus, which causes superficial blisters and scaly crusts. Treatment involves potent topical or intralesional steroids combined with systemic corticosteroids and immunosuppressants like azathioprine or mycophenolate mofetil. Refractory cases can be fatal due to infection, sepsis or organ failure.

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246 views

Pemphigus

This document summarizes information about pemphigus, an autoimmune blistering disease of the skin and mucous membranes. It is caused by IgG antibodies directed against desmoglein proteins in the skin. There are two main types: pemphigus vulgaris, which causes flaccid blisters on skin and mucous membranes, and pemphigus foliaceus, which causes superficial blisters and scaly crusts. Treatment involves potent topical or intralesional steroids combined with systemic corticosteroids and immunosuppressants like azathioprine or mycophenolate mofetil. Refractory cases can be fatal due to infection, sepsis or organ failure.

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iSmayli (smyle-smayl)
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© © All Rights Reserved
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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

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