Cassian Sitaru

..In conclusion, the use of tetrameric NC16A in ELISA results in a sensitive and specific tool for diagnosis and monitoring of BP and PG...

..This animal model for EBA should facilitate further dissection of the pathogenesis of this disease and development of new therapeutic strategies...

..The new mechanistic insights gained from investigating EBA pathogenesis will facilitate the design of immunomodulatory interventions for this and other pathogenetically related organ-specific, antibody-dependent autoimmune diseases...

..This robust experimental system should greatly facilitate further studies on the pathogenesis of EBA and the development of novel immunomodulatory therapies for this and other autoimmune diseases...

..These novel findings shed new light on additional mechanisms of blister formation in pemphigoid diseases and open the way for further informative studies...

..Characterization of the pathogenically relevant subclass(es) of autoantibodies not only provides mechanistic insights, but should greatly facilitate the development of improved therapeutic modalities of autoimmune blistering diseases...

..Understanding the mechanisms by which autoantibodies induce blisters should facilitate the development of more specific therapeutic strategies of autoimmune blistering diseases...

..These findings strongly suggest that elastase and gelatinase B are essential for granulocyte-mediated proteolysis resulting in dermal-epidermal separation in EBA and BP patients' skin...

..Moreover, for the first time, we clearly demonstrate that BP IgG4 autoantibodies have the capacity to induce leucocyte-dependent tissue damage...

..Our data indicate that the Ca(2+)-modulated expression of BP180 is functionally relevant. This finding sheds further light on the complex pathomechanism in blister formation of BP180-related autoimmune blistering skin diseases...

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..These studies establish a robust animal model that will be a useful tool for dissecting the mechanisms of blister formation and will facilitate the development of more effective therapeutic strategies for managing pemphigoid diseases...

..We demonstrate the capacity of autoantibodies to type VII collagen to trigger an Fcgamma-dependent inflammation leading to split formation in cryosections of human skin...

..These findings should greatly facilitate the development of improved disease models and novel therapeutic strategies...

..e. the generation of subclass-specific adsorbers, used for extracorporal immunoapheresis, or the shifting of the autoimmune response to production of non-pathogenic autoantibodies...

..These results indicate that for disease models using the passive transfer of patient autoantibodies, their interaction with the innate factors of the host should be optimized to match the human situation...

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..4 to 5.6. Six different p200-specific sera recognized an identical protein spot of two-dimensionally separated dermal extracts, confirming that patients with this novel autoimmune disease indeed form a single pathobiochemical entity...

..Discontinuation of the antibiotics resulted in a rapid resolution of the skin lesions. This is a most unusual case of localized drug-induced linear IgA disease...

..The aim of our present study was to develop an accurate immunoassay for assessing the presence of autoantibodies against collagen VII in large cohorts of patients and healthy donors...

..Of interest, although the autoimmune response clearly shifted with regard to the target antigens, the patient's autoantibodies against desmosomal and hemidesmosomal components showed the same IgG subclass distribution...

..Our in vitro results support the idea that autoantibodies to bullous pemphigoid 180 from patients with bullous pemphigoid are of pathogenic relevance...

..In addition, the ELISA is useful to monitor autoantibody serum levels. LEVEL OF EVIDENCE: II-2..

..Taken together, these data demonstrate that physiological shedding of collagen XVII generates neoepitopes, which may serve as a target of blister-inducing autoantibodies...

..Therefore, the aim of the present study was to develop an ELISA to detect IgA autoantibodies against collagen XVII in the sera of patients with pemphigoids...

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..Here, we describe a scarring mucocutaneous variant of an autoimmune blistering skin disorder that extends the current clinical and immunopathologic spectrum of this group of diseases...

..The unusual clinical and immunologic profile of this case suggests that molecular specificity of circulating autoantibodies affects the clinical presentation of autoimmune subepidermal blistering skin diseases...

..This study identifies the epitopes relevant for blister induction in PG and should facilitate the development of an antigen-specific immunoadsorption therapy for this disease...

..Therefore, modulation of FcRn function using inhibitors may reduce pathogenic IgG levels, offering therapeutic benefit in patients with antibody-mediated diseases...

..This review article describes the immunopathological features of autoimmune bullous diseases and the immunological and molecular tests used for their diagnosis and monitoring...

..Our findings suggest that the subacute prurigo form of BP is a true variant of BP...

..In this review, the current knowledge on T cell involvement in pemphigus and pemphigoid is summarised...

..Possible applications of this new MAb include antigen mapping in patients with hereditary epidermolysis bullosa and immunoaffinity purification of cell-derived type XVII collagen...

..Thus, this assay meets the demands of a simple and effective diagnostic tool for detecting circulating antibodies against FL-BP180 and may also be used in laboratories without access to molecular biological technology...

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..This ELISA provides a useful tool for the detection of autoantibodies to BP230 in BP and other diseases associated with an autoimmune response to this protein...

..Our data demonstrate a heterogeneous binding pattern of autoantibodies to BP180 NC16A in patients with CP...

..We report a most unusual case of an autoimmune subepidermal blistering with an exclusive IgG3 reactivity to laminin 5...

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..We show that basement membrane levels of collagen VII rise at late embryonic life, concomitant with descending Fras1/Frem immunolabeling...

..Anti-epiligrin cicatricial pemphigoid (AECP) is a subepidermal blistering disease characterized by circulating anti-basement membrane autoantibodies to laminin 5...

..Mucous membrane pemphigoid is a group of autoimmune bullous diseases, mediated by autoantibodies directed against different proteins in the dermoepidermal junction, including the BP180 antigen...

..Based on these results, the diagnosis of an anti-laminin 5 mucous membrane pemphigoid was established. Oral treatment with dapsone, combined with intravenous dexamethasone pulse therapy, led to rapid improvement...