Autoimmune blistering diseases

a. Pemphigus group (diseases with intra-epidermal

blistering)
b. Pemphigoid group (diseases with subepidermal
blistering)
a. Types of pemphigus groups:

Pemphigus vulgaris,
Pemphigus vegetans

Pemphigus foliaceus
Pemphigus erythematosus

Paraneoplastic pemphigus

Drug-induced pemphigus


Acantholytic intraepidermal blistering is produced by autoantibodies against
desmoglein
Dsg1
Dsg 3 Dsg 3
Dsg 1
Ac anti-Dsg 3
Skin
Mucous
membrane
Pemphigus vulgaris


The desmoglein compensation
hypothesis

Dsg1
Dsg 3 Dsg 3
Dsg 1
Ac anti-Dsg 3
skin
Mucous
membrane
Ac anti-Dsg 1
Pemphigus vulgaris

The desmoglein compensation
hypothesis
Pemphigus vulgaris
IFI 320, ELISA dsg1 799,8 dsg 3 53,9

Pemphigus vegetans

Pemphigus foliaceus
IFI 1280 ELISA Dsg3 2557 IFI160 Dsg3 75,5
The clinical phenotype of pemphigus, in particular the severity of cutaneous and
oral lesions, could be determined mainly by the quantities of Dsg1 and Dsg3
autoantibodies.

IIF 640, Dsg1 184, IIF 640 Dsg1 53,8
Dsg3 5732 Dsg3 19,3
IFI 640 Dsg1 377 Dsg 3 7328
+ adenocarcinoma of the colon
IMUNFLUORESCENA DIRECT
IMUNFLUORESCEN INDIRECT
IMUNFLUORESCENA DIRECT I
INDIRECT
ELISA detectarea autoanticorpilor mpotriva dsg 1 i dsg 3
- difereniere ntre pemfigusul vulgar i foliaceu
IgG i C3
IMUNOBLOT
ELISA
AG IMOBILIZAT PE UN SUPORT SOLID
INCUBARE CU SERUL PACIENTULUI
AC SECUNDARI
REACIE DE CULOARE ANALIZAT FOTOMETRIC
a. Pemphigoid group (diseases with subepidermal blistering)

Autoantibodies are produced against hemidesmosome structural proteins
BP180 (type XVII collagen) and BP230.
Autoantibodies against the membrane-proximal NC 16a domain of BP180
play a major role in pathogenesis.
Filamente Filamente de k de keratin eratin
LAMINA LUCIDA
LAMINA DENSA
SUBLAMINA DENSA
BP180
4 I ntegrin
BP230
Plectina
Laminina 5
Colagenul de ti p VII
Desmogleina 1
Desmogleina 3
Desmocolina 1
KERATINOCIT BAZAL
Desmosomul i hemidesmosomul
Lan 4
Lan 6
Bullous pemphigoid
Clinical features:
-pruritic urticarial lesions
-tense large blisters
Histopathology:
-sub-epidermal blister
-superficial dermal infiltrate
Direct immunofluorescence:
-linear deposition of IgG and C3 in the
epidermal basement membrane

IIF ss 640 BP180 727
IIF ss 160 BP180 48,6
Schmidt E. et al. Serum levels of autoantibodies to BP180 correlate
with disease activity in patients with bullous pemphigoid.Arch
Dermatol 2000;136:174-8
Epidermolysis bullosa acquisita
Indirect
immunofluorescence
Direct
immunofluorescence
Linear IgA dermatosis
Dermatitis herpetiformis