this topic is on pemphigus vulgaris. describes in detail regarding the pemphigus, its causes, pathophysiology, management

1. Submitted by-
RUPAM
CHATURVEDI
GROUP –E1
ROLL NO -14
BULLOUS
PEMPHIGO
ID AND
PEMPHIGO
ID
VULGARIS

2. BULLOUS
PEMPHIGOID
 Bullous pemphigoid is
an autoimmune pruritic skin disease ,
aged over 60.
 involve the formation of blisters (bullae)
in the space between
the epidermal and dermal skin layers.
 It is classified as a type II
hypersensitivity reaction, with the
formation of anti-
hemidesmosome antibodies.

3. Itching skin, weeks or
months before blisters
form.
Large blisters that don't
easily rupture when
touched, often along
creases or folds in the
skin
Skin around the blisters
that is normal, reddish
or darker than normal
Eczema or a hive-like
rash
Small blisters or sores
in the mouth or other
mucous membranes
(benign mucous
membrane pemphigoid)

4.  initiated by the formation of IgG autoantibodies targeting dystonin, also called
bullous pemphigoid antigen 1,or type XVII collagen, also called bullous
pemphigoid antigen 2 component of hemidesmosomes.
 immunomodulators results in a variable surge of immune cells,
including neutrophils , lymphocytes and eosinophils coming to the affected area.
 a separation along the dermoepidermal junctionand eventually stretch bullae

5.  1) pruritus and/or predominant cutaneous blisters,
 (2) linear IgG and/or C3c deposits (in an n- serrated
pattern) by direct immunofluorescence microscopy (DIF) on
a skin biopsy specimen.
 (3) positive epidermal side staining by indirect
immunofluorescence microscopy on human salt-split skin.

6.  Pemphigus vulgaris is a rare blistering disease of the skin and mucous
membranes.
 primarily affects older patients with an median age of onset of 71 years of age.
 Blisters commonly erode and leave ulcerated lesions and erosions.
 A positive Nikolsky sign (induction of blistering in normal skin or at the edge of a
blister) is indicative of the disease.

7. Pemphigus is
an autoimmune
disease caused
by antibodies directed
against both desmoglein
1 and desmoglein 3 present
in desmosomes.
Loss of desmosomes results
in loss of cohesion
between keratinocytes in
the epidermis, and a
disruption of the barrier
function served by intact
skin.

8.  Early in the disease patients may have erosions in the mouth or blisters on the
skin..
 the blisters should demonstrate a positive Nikolsky's sign, in which the skin
sloughs off from slight rubbing.
 The gold standard for diagnosis is a punch biopsy from the area around the lesion
that is examined by direct immunofluorescent staining.
 ELISA for desmogleins 3 and 1 are available and may also parallel disease activity

9.  CORTICOSTEROIDS .
 IV IMMUNOGLOBULIN
 MYCOPHENOLATE MOFETIL
 METHOTREXATE
 RTUXIMAB