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Autoimmune dermatoses

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Information

Bullous autoimmune dermatoses are rare, blister-forming diseases of the outer skin and the adjacent mucous membranes. They are characterized 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 localization 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 Guidelines

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 esophagus (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.

Autoimmune dermatoses products

For Research Use Only. Not For Use In Diagnostic Procedures.
The individual product regulatory statements may vary, please refer to the instructions for use for more information.

wdt_ID Method Parameter Substrate Species/ Antigen
1 IFA AB adsorbent
(dermatology IFA)
2 ELISA Dermatology Profile (BP180-NC16A-4X,
BP230-CF, desmoglein 1, desmoglein 3,
envoplakin, collagen type VII separately)
antigen-coated
microplate wells
see individual ELISA
3 ELISA envoplakin antigen-coated
microplate wells
recombinant, expression
in E. coli
4 IFA antibodies against desmoglein 1
(pemphigus-associated)
5 ELISA desmoglein 1 antigen-coated
microplate wells
recombinant, expression in
mammalian cells, extracellular domain
6 IFA desmoglein 1
desmoglein 3
transfected cells
transfected cells
control transfection
(3 BIOCHIPs per field)
EU 90
EU 90
EU 90
7 IFA antibodies against desmoglein 3
(pemphigus-associated)
8 ELISA desmoglein 3 antigen-coated
microplate wells
recombinant, expression in
mammalian cells, extracellular domain
9 IFA antibodies against epidermis: desmosomes
(pemphigus vulgaris control)
10 IFA Dermatology Screen (EM) EUROPattern
epidermis:
prickle cell desmosomes
epidermal basement membrane
oesophagus monkey
11 IFA epidermis:
prickle cell desmosomes
epidermal basement membrane
oesophagus monkey
12 IFA Dermatology Screen 1 EUROPattern
epidermis
epidermis
2 BIOCHIPs per field:
oesophagus
tongue
monkey
monkey
13 IFA epidermis
epidermis
oesophagus
tongue
(2 BIOCHIPs per field)
monkey
monkey
14 IFA 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
15 IFA Dermatology Mosaic 20 EUROPattern
epidermis
pemphigoid antigens
2 BIOCHIPs per field:
oesophagus
salt-split skin
monkey
monkey
16 IFA Dermatology Mosaic 20
epidermis
pemphigoid antigens
2 BIOCHIPs per field:
oesophagus
salt-split skin
monkey
monkey
17 IFA 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
18 IFA antibodies against epidermis: basement membrane
(bullous pemphigoid control)
19 IFA epidermis:
prickle cell desmosomes
epidermal basement membrane
tongue monkey
20 IFA antibodies against BP230
(associated with bullous pemphigoid)
21 ELISA BP230-CF antigen-coated
microplate wells
recombinant, expression in E. coli
22 IFA antibodies against BP180
(associated with bullous pemphigoid)
23 ELISA BP180-NC16A-4X antigen-coated
microplate wells
recombinant, expression in E. coli,
tetramer of the NC16A domain
24 IFA keratin
(filaggrin, RA keratin)
oesophagus rat
25 IFA transitional epithelium
(detection of paraneoplastic pemphigus)
bladder mucosa rat
Method Parameter Substrate Species/ Antigen
Contact our Autoimmune Product Team for further details and information
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