Autoimmune bullous skin diseases are characterized by autoantibodies against adhesion molecules of the skin. Pemphigus is a disorder with an intraepidermal loss of adhesion and is characterized by fragile blisters and erosions. Pemphigus vulgaris often shows extensive lesions of the oral mucosa, while pemphigus foliaceus is commonly restricted to cutaneous involvement with puff pastry-like scale formation. Paraneoplastic pemphigus is obligatorily associated with malignancies and often presents as hemorrhagic stomatitis with multiforme-like exanthems. IgA pemphigus typically presents with pustules and annular plaques but not with mucosal involvement. The clinical spectrum of the pemphigoids includes tense blisters, urticarial plaques, and prurigo- like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a "cluster of jewels"-like pattern in childhood and is more heterogeneous in adulthood. Classical epidermolysis bullosa acquisita shows extensive skin fragility. Dermatitis herpetiformis is associated with gluten-sensitive enteropathy and manifests clinically with severe itching and papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The intention of the review is to demonstrate the heterogeneous clinical spectrum of autoimmune bullous disorders.
Autoimmune bullous skin diseases represent a heterogenous group of disorders of skin and mucosa which are commonly associated with IgG or IgA autoantibodies against distinct adhesion molecules of the skin. The antibodyinduced loss of adhesion between epidermis and dermis results in blister formation and extensive erosions. There is a great need for rapidly establishing the diagnosis of these disorders since they may run a severe and potentially life-threatening course. In addition, because of their rarity and heterogeneous symptoms, autoimmune bullous skin diseases often pose a major diagnostic challenge. While histopathological examinations provide evidence for the level of blister formation, immunofluorescence microscopy has been established to identify tissue-bound and circulating autoantibodies. Direct immunofluorescence microscopy represents the gold standard for detecting tissue-bound autoantibodies. Indirect immunofluorescence microscopy with defined tissue substrates is considered the first step in detecting circulating autoantibodies. Confirmatory tests such as ELISA, immunoblot or immunoprecipitation analyses are performed utilizing recombinant proteins or keratinocyte extracts. The later assays can be used for primary diagnosis as well as for immunoserological follow-up. Systemic immunosuppressive drugs usually represent the main therapeutic regimen. Initially, systemic corticosteroids are commonly administered in combination with steroid-sparing, immunosuppressive agents. Novel targeted treatments such as immunoadsorption, rituximab or high-dose intravenous immunoglobulins have proven to be highly effective in severe and refractory pemphigus. This review presents a state-of-the-art algorithm for making the diagnosis of autoimmune bullous disorders and provides an overview on currently available therapeutic options.
The present findings suggest that IgG reactivity against BP230 (i.e. the COOH terminus), and to a lesser extent against BP180, is a common finding in pruritic disorders of the elderly with a wide clinical spectrum. IgG-mediated autoimmunity against the intracellular BP230 may facilitate a chronic, inflammatory response eventually leading to full-blown BP which is presumably associated with IgG against BP180.
Background: Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering skin disease which is associated with pathogenic IgG autoantibodies against desmogleins (Dsg) 1 and 3. Novel therapeutic strategies such as immunoadsorption (IA) or the anti-CD20 antibody rituximab (Rtx) hold promise to be effective in severe or recalcitrant PV. Patients and Methods: In the present retrospective study, 6 patients with extensive cutaneous PV were subjected to adjuvant IA treatment while 5 patients with severe mucosal PV received adjuvant Rtx treatment. Results: Within 6 months, IA and Rtx induced excellent clinical responses which were associated with a significant reduction of prednisolone doses and a decrease in anti-Dsg-specific IgG. Over a 12-month period, 3 IA-treated patients required additional adjuvant drugs while all of the PV patients on Rtx had no or only minimal residual symptoms. Conclusion: The relative therapeutic (long-term) efficacy of IA and Rtx in cutaneous versus mucosal PV needs to be evaluated in a prospective study.
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