Blistering Diseases

8/11/2019 Blistering Diseases

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Autoimmune blistering diseases

a.Pemphigus group (diseases with intra-epidermal

blistering)

b.Pemphigoid group (diseases with subepidermal

blistering)


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a. Types of pemphigus groups:

Pemphigus vulgaris,

Pemphigus vegetans

Pemphigus foliaceus

Pemphigus erythematosus

Paraneoplastic pemphigus

Drug-induced pemphigus


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Acantholytic intraepidermal blistering is produced by autoantibodies against

desmoglein


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Dsg1

Dsg 3Dsg 3

Dsg 1

Ac anti-Dsg 3

Skin Mucousmembrane

Pemphigus vulgaris

The desmoglein compensationhypothesis


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Dsg1

Dsg 3Dsg 3

Dsg 1

Ac anti-Dsg 3

skin Mucousmembrane

Ac anti-Dsg 1

Pemphigus vulgaris

The desmoglein compensationhypothesis


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Pemphigus vulgaris


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IFI 320, ELISA dsg1 799,8 dsg 3 53,9


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Pemphigus vegetans


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IFI 1280 ELISA Dsg3 2557 IFI160 Dsg3 75,5The 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.


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IIF 640, Dsg1 184, IIF 640 Dsg1 53,8

Dsg3 5732 Dsg3 19,3


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IFI 640 Dsg1 377 Dsg 3 7328

+ adenocarcinoma of the colon


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IMUNFLUORESCENA DIRECT

IMUNFLUORESCEN INDIRECT


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IMUNFLUORESCENA DIRECTIINDIRECT

ELISA detectarea autoanticorpilor mpotriva dsg 1 i dsg 3

- difereniere ntre pemfigusul vulgar i foliaceu

IgG i C3


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IMUNOBLOT

ELISA

AGIMOBILIZAT PE UN SUPORT SOLIDINCUBARE CU SERUL PACIENTULUI

AC SECUNDARI

REACIE DE CULOARE ANALIZAT FOTOMETRIC


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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.


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FilamenteFilamente de kde keratineratin

LAMINA LUCIDA

LAMINA DENSA

SUBLAMINA DENSA

BP180 4 Integrin

BP230 Plectina

Laminina 5

Colagenul de tip VII

Desmogleina 1

Desmogleina 3

Desmocolina 1

KERATINOCIT BAZAL

Desmosomul i hemidesmosomul

Lan4

Lan 6


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IIF ss 640 BP180 727

IIF ss 160 BP180 48,6Schmidt E. et al. Serum levels of autoantibodies to BP180 correlate

with disease activity in patients with bullous pemphigoid.ArchDermatol 2000;136:174-8

E id l i b ll i it


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Epidermolysis bullosa acquisita


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Indirect

immunofluorescence

Direct

immunofluorescence

Linear IgA dermatosis


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Linear IgA dermatosis

Dermatitis herpetiformis


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Dermatitis herpetiformis

Blistering Diseases

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