DIAGNOSTIC TEST PANEL 550
Bullous Dermatosis
Diagnostic test panel for antibodies against Envoplakin (IgG), Collagen VII (IgG), BP180 (IgG), BP230 (IgG), and Desmoglein 1 & 3 (IgG). For suspicion of autoimmune skin disease, Bullous Pemphigoid (BP), Pemphigus, Paraneoplastic Pemphigus, and Epidermolysis Bullous Aquisita (EBA).
Indication
Suspicion of autoimmune skin disease Bullos Pemfigoid (BP), Pemfigus, Paraneoplastic Pemfigus and Epidermolysis Bullos Aquisita (EBA). Differential diagnosis and disease monitoring.
Clinical background
Autoimmune bullous skin diseases include a group of serious diseases with blisters and erosions on the skin and mucous membranes. The skin has adhesion molecules that stick the keratinocytes to each other and to the underlying basement membrane. Several of its molecules are the targets of autoimmune skin diseases. An important factor is circulating antibodies directed against adhesion structures in the epidermis, the basement membrane, or anchoring fibrils of the dermis. Pemphigus and pemphigoid are the two most common and most serious bullous diseases, and the autoantibodies are considered an important part of the pathogenesis. In pemphigus, the antibodies are directed against desmosomal adhesion proteins in the epidermis, whereas hemidesmosomal proteins are the targets of the antibodies in pemphigoid and similar diseases, such as IgA bullous dermatosis. In epidermolysis bullosa acquisita (EBA), anchoring fibrils (type VII collagen) are the antigen.
Pemphigus
Pemphigus is a group of life-threatening autoimmune bullous skin diseases with intraepithelial blisters. The molecular background of the blistering is the interference of the adhesion between the keratinocytes (acantholysis) by the autoantibodies against intercellular adhesion structures. Various forms of pemphigus have been identified, and they have intraepidermal divisions on different levels of the skin. In pemphigus vulgaris (PV), the blisters are located in the deeper layers of the epidermis, whereas in pemphigus foliaceus (PF), a less serious form, they are located in the upper layers of the epidermis. In PV, autoantibodies are found against the extracellular domain of desmoglein 3, an adhesion molecule found on the epidermal keratinocytes. Even though desmoglein 3 is the most important antigen in PV, many patients may also have antibodies against desmoglein 1, which is the most important antigen in PF. The autoantibodies in pemphigus are polyclonal and most of them are of the IgG4 subclass in active disease, whereas in remission they are usually IgG1.
Pemphigoid
Bullous pemphigoid (BP) is an autoimmune bullous skin disease that is recognized by subepidermal bullae and circulating antibodies against the basement membrane zone in the epithelium. Earlier studies have shown that these autoantibodies primarily react with two components of hemidesmosomes, BP180 (type XVII collagen) and BP230. Hemidesmosomes are multiple protein complexes, which participate in the epithelial cell adhesion to the underlying basement membranes and bind the cytoskeleton to the extracellular matrix. BP230 is cytoplasmic and binds to intermediary filaments. BP180 is a transmembrane protein that can bind to receptors in the extracellular matrix. Antibodies against BP180 induce blisters if injected in mice. This shows that the autoantibodies against BP180 are pathogenic and are responsible for producing the blisters in BP. It has been shown that the NC16 domain, which is closest to the cell membranes, contains most of the epitopes of the molecule. There are several variants of BP that have antibodies against BP180 but that are clinically distinct from BP. Pemphigoid gestationis (PG) is a bullous disease that occurs in the last trimester of pregnancy, and that also has antibodies against BP180. Cicatricial pemphigoid (CP) is a subepidermal autoimmune bullous disease, which, in contrast to BP, mainly affects mucous membranes in the conjunctive and oral, perianal, and genital mucous membranes and leads to scarring in the affected tissue. In CP, it has been shown that the antibodies react with several antigens besides B180, e.g., laminin 5.
Desmoglein 1 and 3 IgG antibodies
ELISA with recombinant antigens desmoglein 1 and 3. Desmoglein 1 is a desmosomal cadherin with a molecular weight of 160kD, whereas desmoglein 3 has a molecular weight of 130kD.
In pemphigus foliaceus the antibodies are mainly directed against desmoglein 1. In pemphigus vulgaris the antibodies are mainly directed against the extracellular domain of desmoglein 3 but some patients may also have antibodies against desmoglein 1.
BP180 and BP230 IgG antibodies
Serum samples are analyzed by ELISA technique with recombinant BP180 and BP230. BP180 has a molecular weight of 180kD and is also called BPAG2. BP230 has a molecular weight of 230kD and is also called BPAG1.
Antibodies towards BP180 and BP230 occur in patients with bladder disease Bullous pemphigoid (BP). The antibodies are directed against the two components of the basement membrane zone of the epithelium BP180 (type XVII collagen) and BP230. Anti-BP230 is found in approximately 65% of patients with BP, while anti-BP180 is found in almost all patients.
The serum level of anti-BP180 correlates with disease activity, and BP230 correlates with disease duration. However, it seems that anti-BP230 does not cause blisters but is rather a consequence of damage to the keratinocytes. The antibodies may also be associated with paraneoplastic pemphigoid and other variations of BP. The different variants of BP include Pemphigoid gestationis (PG), which can develop during pregnancy, and Cicatricial pemphigoid (CP), which is a sub-epidermal autoimmune bladder disease.
Envoplakin IgG antibodies
Detection of Anti-Envoplakin (IgG) antibodies are intended for diagnostic of PNP (paraneoplastic pemphigus) which is a serious dermatosis associated with an underlying malignant disease - especially lymphoproliferative diseases non-Hodgkin's lymphoma and CLL (chronic lymphocytic leukemia). PNP can occur prior to the manifestation of malignancy why suspected PNP always is followed by investigation of malignant disease. Antibody concentrations correlate with disease activity and may be used to monitor treatment efficacy.
Collagen type VII IgG antibodies
These antibodies occur in epidermolysis bullosa acquisita (EBA).
References
- Probst C. et al., 2009. Clin Chim Acta. Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus.
- Baum S. et al, 2014. Autoimmun Rev. Diagnosis and classification of autoimmune blistering diseases.
- Schmidt E. and Zillikens D., 2011.Dtsch Arztebl Int. The diagnosis and treatment of autoimmune blistering skin diseases.
Tests included in panel
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How to order
This test panel is available worldwide for hospitals, clinics, and physicians.
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Print and complete the request form
Download the request form. Clearly state the name and phone number of the referring hospital, clinic, or physician. -
Prepare your samples
Serum: At least 0.5 mL serum (plain serum tubes without additives).
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Send samples and request form
Within Sweden
Samples can be sent at room temperature to:
Envelopes and smaller boxes:
Wieslab AB, Box 50117, 20211 Malmö, Sweden
Larger boxes and frozen samples:
Wieslab AB, Lundavägen 151, 21224 Malmö, Sweden
International
Samples can be sent at room temperature to:
Wieslab AB, Lundavägen 151, 21224 Malmö, Sweden
Last updated: 2025-08-18
