Pemphigus and Pemphigoid

Question 1

what happens if pemphigus is left untreated

Answer 1

can be fatal as a result of extensive skin/mucosal damage leading to fluid and electrolyte loss and infection

Question 2

pathogenesis of pemphigus

Answer 2

auto immune reaction
IgG auto-antibodies target desmogleins 1 and 3, which are adhesion proteins in desmosomes of stratified squamous epithelia

Question 3

what does pemphigus affect

Answer 3

mucous membranes and skin

Question 4

clinical signs and symptoms of pemphigus

Answer 4

thin roofed vesicles or bullae that rupture leaving erosions and ulceration
oral mucous membranes often FIRST AFFECTED

Question 5

histopathology of PV

Answer 5

suprabasal cleft formation
intraepithelial vesicles containing free floating acantholytic cells

Question 6

immunofluorescence of PV

Answer 6

DIF shows intracellular deposition of IgG in epidermis
BASKET WEAVE PATTERN

Question 7

management of PV

Answer 7

referral to OM
n.b. ulceration tx
systemic steroids are mainstay of tx
azathioprine or mycophenolate may be used

Question 8

MMP pathogenesis

Answer 8

antibodies target components of basement membrane zone BP180 and BP230

Question 9

what does MMP affect

Answer 9

primarily mucous membranes, less commonly skin

Question 10

clinical signs of MMP

Answer 10

subepidermal blisters that are more stable and less prone to rupture than pemphigus
gingivae often affected

Question 11

histology of MMP

Answer 11

subepidermal blistering

Question 12

immunofluorescence of MMP

Answer 12

LINEAR deposition of IgG and C3 along basement membrane

Question 13

management of MMP

Answer 13

referral to OM and ophthalmologist if ocular involvement
steroids
azathioprine and mycophenolate