Autoimmune dermatoses

Clinical information

Bullous autoimmune dermatoses are rare, blister-forming diseases of the outer skin and the adjacent mucous membranes. They are characterised by the formation of autoantibodies against structural proteins of the skin. These structural proteins establish the cell-to-cell contact in keratinocytes within the epidermis and the adhesion of the epidermis to the dermis. Bullous autoimmune dermatoses are divided into four main groups based on their target antigens and the localisation of the blisters:

  • Pemphigus diseases: desmoglein 1 (Dsg1), desmoglein 3 (Dsg3), different plakins (mostly envoplakin)
  • Pemphigoid diseases: BP180, BP230, laminin 332
  • Epidermolysis bullosa acquisita: collagen type VII
  • Dermatitis herpetiformis: endomysium (tissue/epidermal transglutaminase), deamidated gliadin peptides (GAF-3X)

Diagnostics

A conclusive diagnosis of blister-forming autoimmune dermatoses requires the detection of both tissue-bound autoantibodies by direct immunofluorescence and circulating autoantibodies. The circulating specific autoantibodies against epidermal antigens (prickle cell desmosomes and epidermal basement membrane) in patient serum are detected using the indirect immunofluorescence test (IIFT) with tissue sections of primary oesophagus (or tongue). For further differentiation of autoantibodies against basement membrane structures, tissue sections of primate salt-split skin are used. Final diagnosis is based on a combination of the clinical picture with the detection of autoantibodies against the individual target antigens using IIFT, monospecific ELISA or immunoblot analyses.

Patients who suffer from bullous pemphigoid (BP) exhibit autoantibodies against BP180 and frequently also against BP230. The serum level of autoantibodies against BP180 correlates with the disease activity of BP, the serum level of autoantibodies against BP230 with the duration of the disease. Hence, the Anti-BP180-NC16A-4X ELISA (IgG) and the Anti-BP230-CF ELISA (IgG) are not only suited to reliably serologically identifying BP, but also to monitoring the activity of the disease before and during treatment and assessing the disease duration. Autoantibodies against desmoglein 1 and 3 are markers for pemphigus diseases. IIFT has proven valuable for detecting circulating autoantibodies in pemphigus. ELISA using recombinant desmoglein 1 and 3 offer the same sensitivity and specificity as IIFT. The anti-Dsg1 and -Dsg3 antibody levels measured correlate to a large extend with the severity and activity of the disease and the therapy success. The determination of autoantibodies against envoplakin contributes to diagnosis of PNP as well as differential diagnostic clarification. The determination of autoantibodies against collagen type VII confirms the diagnosis of EBA and enables the delimitation from other bullous autoimmune dermatoses.

Selected Products

Method
Parameter
Substrate
Species
IIFT
AB adsorbent
(dermatology IIFT)
ELISA
Dermatology Profile (BP180-NC16A-4X,
BP230-CF, desmoglein 1, desmoglein 3,
envoplakin, collagen type VII separately)
antigen-coated
microplate wells
ELISA
envoplakin
antigen-coated
microplate wells
IIFT
antibodies against desmoglein 1
(pemphigus-associated)
ELISA
desmoglein 1
antigen-coated
microplate wells
IIFT
desmoglein 1
desmoglein 3
transfected cells
transfected cells
control transfection
(3 BIOCHIPs per field)
EU 90
EU 90
EU 90
IIFT
antibodies against desmoglein 3
(pemphigus-associated)
ELISA
desmoglein 3
antigen-coated
microplate wells
IIFT
antibodies against epidermis: desmosomes
(pemphigus vulgaris control)
IIFT
epidermis:
prickle cell desmosomes
epidermal basement membrane
oesophagus
monkey
IIFT
Dermatology Screen (EM) EUROPattern
epidermis:
prickle cell desmosomes
epidermal basement membrane

oesophagus

monkey
IIFT
epidermis
epidermis
oesophagus
tongue
(2 BIOCHIPs per field)
monkey
monkey
IIFT
Dermatology Screen 1 EUROPattern
epidermis
epidermis
2 BIOCHIPs per field:
oesophagus
tongue

monkey
monkey
IIFT
Dermatology Mosaic 11
epidermis
pemphigoid antigens
transitional epithelium
desmoglein 1
desmoglein 3
BP230gC

BP180-NC16A-4X
gliadin (GAF-3X)
cell nuclei (ANA)
endomysium
11 BIOCHIPs per field:
oesophagus
salt-split skin
bladder mucosa
transfected cells
transfected cells
transfected cells
control transfection
BP180-NC16A-4X BIOCHIPs
gliadin (GAF-3X) BIOCHIPs
HEp-2 cells
liver

monkey
monkey
rat
EU 90
EU 90
EU 90
EU 90


human
monkey
IIFT
Dermatology Mosaic 20
epidermis
pemphigoid antigens
2 BIOCHIPs per field:
oesophagus
salt-split skin

monkey
monkey
IIFT
Dermatology Mosaic 20 EUROPattern
epidermis
pemphigoid antigens
2 BIOCHIPs per field:
oesophagus
salt-split skin

monkey
monkey
IIFT
Dermatology Mosaic 7
epidermis
pemphigoid antigens
BP230gC
desmoglein 1
desmoglein 3
BP180-NC16A-4X
6 BIOCHIPs per field:
oesophagus
salt-split skin
transfected cells
transfected cells
transfected cells
BP180-NC16A-4X BIOCHIPs

monkey
monkey
EU 90
EU 90
EU 90
IIFT
antibodies against epidermis: basement membrane
(bullous pemphigoid control)
IIFT
epidermis:
prickle cell desmosomes
epidermal basement membrane
tongue
monkey
IIFT
antibodies against BP230
(associated with bullous pemphigoid)
ELISA
BP230-CF
antigen-coated
microplate wells
IIFT
antibodies against BP180
(associated with bullous pemphigoid)
ELISA
BP180-NC16A-4X
antigen-coated
microplate wells
IIFT
keratin
(filaggrin, RA keratin)
oesophagus
rat
IIFT
transitional epithelium
(detection of paraneoplastic pemphigus)
bladder mucosa
rat
IIFT
antibodies against keratin
(filaggrin ab control)
IIFT
pemphigoid antigens
salt-split skin
monkey
IIFT
laminin 332 (LAM332)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
antibodies against collagen type VII
(associated with epidermolysis bullosa aquisita)
ELISA
collagen type VII
antigen-coated
microplate wells
IIFT
collagen type VII NC1
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
ELISA
MabTrack level adalimumab
ELISA
MabTrack anti-drug antibody adalimumab
ELISA
MabTrack level infliximab
ELISA
MabTrack anti-drug antibody infliximab
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