Friday, 11 May 2012

Autoimmunity - Skin Blistering Disease

Pemphigus is an autoimmune blistering disease that targets skin and mucous membrane, caused by autoantibodies to cell surface antigens of stratified epithelia of mucous membranes and skin. These antibodies bind to calcium dependent adhesion molecules in cell surface desmosomes, notably desmoglein 1 (Dsg1) in PF and desmoglein 3 (Dsg3) in PV. Pemphigus patients with both mucosal and skin lesions have antibodies to both Dsg1 and Dsg3.

Bullous Pemphigoid (BP) is an autoimmune disease of the skin and mucous membranes. Skin lesions of BP patients show separation of basal keratinocytes from the underlying dermis leading to subepidermal blisters. Patients with BP have circulating IgG autoantibodies against components of hemidesmosome, and target two distinct molecules, BP230 and BP180. BP180 is considered the target molecule to which pathogenic IgG autoantibodies bind.

Epidermolysis Bullosa Acquisita (EBA) is a chronic subepidermal blistering disease of the skin and mucous membranes. EBA is characterised by the presence of (IgG) antibodies targeting the NC1 domain of Type VII collagen. MBLI has developed a highly specific, highly sensitive ELISA kit for the detection of the NC1 and NC2 domain of Type VII Collagen. 

Related products from MBL International:
MESACUP Desmoglein Test Dsg1 (Product Code: RG-7680EC-D)
MESACUP Desmoglein Test Dsg3 (Product Code: RG-7685EC-D)
BP180 ELISA Kit (Product Code: RG-7695-EC-D)
BP230 ELISA Kit (Product Code: RG-7613EC-D)
Anti-Type VII Collagen ELISA Kit (CE Mark) (Product Code: RG-7845E)

Take a look at MBL’s handy new reference guide here.

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