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Epidermolysis Bullosa
Among the topics discussed are molecular and cell biology, epidemiology, diagnosis, classification, medical and surgical treatments, and clinical outcomes.
Life with Epidermolysis Bullosa (EB)
Epidermolysis bullosa (EB) is a heterogenous group of genodermatoses characterized by the formation of blisters and erosions on skin and mucous membrans from birth on. The cause are mutations in the genes of structural proteins of the junction between epidermis and dermis. This book deals with the treatment of this skin disease itself and its many extracutaneous complications. There is no previous book which has been focused on the therapy and it will be based on evidence-based data derived from the world ́s largest cohort of inherited EB-patients, the American EB Registry. An important chapter will discuss gene therapy in hereditary EB which has been recently successfully performed within a localized skin site on a single EB patient as a proof-of-principle test. Given its unique collective contents, the monograph will provide the primary source for clinical informations of this oftentimes severe multiorgan disease.
Blistering Diseases
This book is a comprehensive compendium of current knowledge on inherited and autoimmune blistering diseases that relates advances in our understanding of the pathogenetic mechanisms to management of the individual diseases. The aim is to provide a detailed reference for dermatologists who care for patients with these conditions and a useful “one-stop information shop” for specialists outside of dermatology. The book opens by describing the structure and biology of the epidermis and basement membrane zone and discussing the genes and proteins that are targets for mutations and autoantibodies. The role of the various diagnostic tests is explained, and clinical manifestations of the specific diseases are presented with the aid of many high-quality illustrations. The forms of treatment appropriate in specific conditions are then described in depth, with coverage of dressings, drugs, surgical procedures, gene therapy, and other novel approaches. Helpful algorithms are included both for testing and monitoring and for treatment.
Epidermolysis Bullosa
Because skin blisters are the initial manifestation of epidermolysis bullosa (EB), patients invariably present to the dermatologist for diagnosis and treatment. However, EB is a systemic disease whose management requires input from clinicians in virtually all fields of medicine, including pediatri cians, surgeons, dentists, gastroenterologists, hematologists, otorhinolaryn gologists, dietitians, and physical therapists, to name a few. Because EB is a rare disease, few clinicians are familiar with it, and many recoil at the pros pect of caring for individuals covered with blisters caused by a disease they know little about. For patients, insult is thus added to injury and they feel abandoned,...
Journal of the House of Representatives of the United States
Some vols. include supplemental journals of "such proceedings of the sessions, as, during the time they were depending, were ordered to be kept secret, and respecting which the injunction of secrecy was afterwards taken off by the order of the House".
