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Hematopoietic Stem Cell Transplantation in Patients with Autoimmune Bullous Skin Disorders
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Pemphigus vulgaris, like all diseases in the pemphigus group, is pathologically characterized by intraepidermal suprabasal acantholytic blisters with detachment of adjacent epidermal keratinocytes. The diagnosis of pemphigus is confirmed by direct immunofluorescence, which shows IgG deposited on the surface of keratinocytes in and around lesions. Anti-desmoglein antibodies can also be detected using ELISA methods in 80-90% of patients.
A comprehensive summary of disease variants implicated in metal allergy
Published in Journal of Toxicology and Environmental Health, Part B, 2022
The term ‘bullous autoimmune dermatoses’ comprises several disease subtypes with shared but distinctive pathophysiological characteristics – the two most common of which are pemphigus and pemphigoid. In both of these diseases, autoantibodies are involved in blistering eruptions of the skin and oral mucosa. Pemphigus-type diseases involve the development of autoantibodies reactive toward desmogleins – proteins involved in cell-cell adhesion – which results in the loss of keratinocyte structural integrity within the epidermis and subsequent lesion formation (Hammers and Stanley 2016). Comparatively, pemphigoid-type conditions emerge in response to autoantibody formation wherein reactivity to hemidesmosomes – proteins that mediate cell adhesion to the basement membrane – results in fixation of complement and subsequent inflammation and lesion emergence (Hofmann, Juratli, and Eming 2018). Both diseases primarily implicate IgG isoforms of effector autoantibodies, however, IgA-mediated variants of both disease types also exist (Kasperkiewicz et al. 2017).