Autoimmune Bullous Diseases

image of desmosomes Autoimmune bullous diseases are a group of rare, acquired disorders characterized by overlapping features, resistance to treatment, and potential lethality. Early descriptions of these diseases focused on their clinical presentations, resulting in significant confusion in both nomenclature and prognosis. The advent of light and electron microscopy added greatly to our ability to accurately characterize these diseases, followed by advances in immunology in the early 1960s that clearly demonstrated the autoimmune nature of many severe blistering diseases. Over the past five decades, research initiatives in laboratories worldwide have yielded notable advances in our understanding of these diseases, including their nosology, pathophysiology, associations, and epidemiology. These advances were based almost exclusively on the study of affected patients (i.e., through applied translational research). Studies showing that autoantibodies were specific for a given disease allowed such immunoreactants to serve as disease "markers" as well as probes for the recovery, characterization, and cloning of corresponding target autoantigens. Interestingly, such target autoantigens typically represent important structural proteins in skin that mediate either cell-cell or cell-matrix adhesion—vital biological processes that have been shown to be directly impaired by the effect of patients' autoantibodies. Immunobullous disorders are among the best understood autoimmune diseases in humans. Basic and translational research in this field has expanded our understanding of autoimmunity and fostered the application of new and advanced therapies. The articles review the subject in detail and provide insights about the future of research in this and related content areas.

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Cutaneous biology is the study of cellular and molecular biology of human skin and its associated diseases. Milestones in Cutaneous Biology brings together highly respected researchers to write on a number of subjects relating to this field's highlights over the last 100 years. Some of the subjects this series will cover are: Desmosome, Basement Membrane, Hair/Sebaceous Glands/Nails, Dermal Proteins/Matrix/Collagen, Wound Healing, Melanocytes/Melanogenesis, Psoriasis, and Atopic Dermatitis.


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