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Autoimmune dermatoses

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Disease information

Autoimmune dermatoses are blister forming diseases of the skin and the associated mucous membranes. They involve formation of autoantibodies against the structural proteins in the skin; these proteins are responsible for cell to cell contacts and the overall integrity of both the epidermis and dermis of the skin. Objectively, we divide such diseases into four categories based on the location of the blisters and the target antigens involved. The types are as follows:

  • 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

The diagnosis of autoimmune dermatoses depends upon the detection of autoantibodies, both tissue-bound and circulating. For circulating antibodies, the patient’s serum is looked up for antibodies against epidermal antigens (prickle cell desmosomes and epidermal basement membrane antigen), using the indirect immunofluorescence (IFA) technique. To narrow down the spectrum, autoantibodies are also detected using tissue sections; those from the esophagus and primate salt-split skin. Final diagnosis stems from a combination of clinical presentation and the detection of antibodies using IFA, monospecific ELISA and immunoblot assays.

Bullous pemphigoid

Patients with bullous pemphigoid come out positive for antibodies against BP180 and BP230. While those against BP180 correlate with the disease activity, antibodies against BP230 correlate with the duration of the disease. Thus, these antibodies not only help make a diagnosis but also help monitor the disease activity before and after the treatment. Also, they help to make a statement on disease duration.

Pemphigus Diseases

Pemphigus diseases involve antibodies against desmoglein 1 and 3. High titers for these antibodies detected on IFA and ELISA (same sensitivity and specificity for both tests) are diagnostic of the disease.

Epidermolysis Bullosa Acquisita

For EBA, high levels of antibodies against collagen type 7 are observed. These antibodies play a dual role; they allow for both disease diagnosis and help differentiate it from other types of autoimmune dermatoses.

Autoimmune dermatoses products

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
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