Autoimmune Blistering Diseases

Autoimmune Blistering Diseases (ABDs) are a group of disorders associated with autoantibodies that are directed against desmosomal structural proteins (Pemphigus) or hemidesmosomal proteins (Bullous Pemphigoid and Epidermolysis Bullosa Acquisita). MBL International offers ELISA kits for detection and monitoring of ABDs. These products are only available in the EU.

Pemphigus is a group of autoimmune blistering diseases of the skin and mucous membranes which are characterized histologically by intraepidermal blisters due to acantholysis (i.e., disruption of the intercellular connections between keratinocytes of the epidermis) and immunopathologically by in vivo bound and circulating immunoglobulin G (IgG) antibodies directed against the cell surface of keratinocytes. The target antigens in pemphigus are Dsg1 and 3, members of the cadherin super family. Pemphigus can be classified into pemphigus vulgaris (PV), pemphigus foliaceus (PF), paraneoplastic pemphigus (PNP), and others. The blisters in PV and PF occur in the deeper region of the epidermis (just above the basal layer) and the upper layer respectively.

Pemphigoid is a group of diseases characterized histologically by subepidermal blisters and immunopathologically by linear deposition of IgG and complement C3 at basement membrane zone (BMZ) in the skin from patients with circulating IgG against the molecules within the dermal-epidermal junction (DEJ). The target antigens recognized by autoantibodies in patients with bullous pemphigoid (BP) are BP180 (a 180 kDa transmembrane protein), and BP230 (a 230 kDa intracellular protein). The target antigens recognized by autoantibodies in other diseases of this group include type VII collagen in epidermolysis bullosa acquisita (EBA) and others. The skin lesions of BP are characterized by tense blisters with significant pruritus. Histopathological analysis detects subepidermal blisters and superficial dermal infiltrations of eosinophils, lymphocytes, and macrophages.

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