Autoimmune Bullous Diseases

Autoimmune bullous diseases are a group of rare skin diseases characterized by intraepidermal and subepidermal bullae formations due to autoantibodies directed against the structural proteins of the epidermis or the dermal-epidermal junction. Early and correct diagnosis and adequate treatment of autoimmune bullous diseases are important as they cause morbidity and mortality in the affected patients. This book, which gives detailed information about autoimmune bullous diseases, has two sections and nine chapters with sixteen contributing authors. The first section describes the structure and tasks of desmosomes and basement membrane zone, which consist of the major antigens of skin integrity targeted by autoantibodies. The second section is about the epidemiology, etiopathogenesis, mucocutaneous, histopathologic and laboratory findings, and therapy of autoimmune bullous diseases.

This book focuses on helping both dermatologists and non-dermatologists approach the treatment of each disease in a pragmatic manner. Each chapter reviews the current literature of treatments for each disease, rating disease severity, and provides pragmatic guidelines based on disease severity on the usage of available medications. The current literature does not provide effective guidelines. This book addresses this deficiency. This book will help guide clinicians in implementing appropriate treatments based on the specific disease and its severity, and possible alternatives in an algorithmic format. It will make the management of these diseases more pragmatic based on both the literature and clinical experience of experts.

Autoimmune disorders of the skin remain an enigma for many clinicians and scientists not familiar with these mostly severe and chronic diseases. The book provides an overview and the latest information on the broad spectrum of cutaneous autoimmune disorders for clinicians, scientists and practitioners in dermatology, medicine, rheumatology, ENT, pediatrics and ophthalmology. The book is unique since it presents the state-of-the-art knowledge on pathophysiology, clinical diagnosis and management of these disorders provided by the world experts in the field. The primary intention is to broaden the understanding of the pathophysiology of cutaneous autoimmune disorders and to provide a practical guide to how to identify and handle these conditions. The book is illustrated with many tables, illustrative figures and clinical color photographs. The third edition has been thoroughly updated and extended by chapters on paraneoplastic cutaneous syndromes, atopic dermatitis and autoimmunity and Skin manifestations of rheumatic diseases.

Pemphigus vulgaris (PV) is the most frequent condition inside the pemphigus group, especially in Europe and North America. It is the prototype of a genetically mediated disease. The HLA alleles associated with PV are well-known. Recent studies found an association between disease severity and some HLA DRB1 and DQB1 alleles. The author of this book discusses the management of PV that should be started with an evaluation of disease severity.

​This book provides obligatory study material on courses on autoimmune bullous diseases. Residents and researchers in dermatology and adjacent disciplines can use the book as a quick reference guide when dealing with these patients. The book contains protocols and follows international guidelines.​ Currently there is no quick primer on Autoimmune Bullous Diseases for dermatologists who wish to learn the subtleties of diagnosis and management of this group of mucocutaneous diseases. Since mucous membranes are also included the following medical disciplines might be interested too: oral medicine and stomatology, ENT, ophthalmology, gynaecology, gastro-enterology. The Center for Blistering Diseases in Groningen has organized since the year 2000 an annual course on Bullous Diseases for dermatologists and pathologists. The staff is selected from the faculty. The course is interactive and problem-oriented. The proposed book would represent the documented practical knowledge of the staff on these diseases. The information should be clear and practical with examples and questions for self-assessment.

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.

This book presents the nuances of dermatology from the African diaspora and the tropics. It not only addresses the dark pigmentation of the patient’s skin and the occurrence of tropical infections, but also the socioeconomic conditions which lead to unique features and the development of skin diseases. Chapters present numerous dermatological cases, with clear/relevant pictures, to serve as illustration of how skin conditions present in African/dark skin. Of these specific conditions, the book includes chapters on eczema, bullous diseases, hair disorders, acne, and papulosquamous disorders. Additionally, chapters address emotionally sensitive and socioeconomic-related issues such as skin bleaching and dermatological manifestations of HIV/AIDS, an infectious condition that disproportionately affects those residing in sub-Saharan Africa. Expertly written text is supplemented by hundreds of high-quality, real patient photos. Written by doctors living and treating patients in the tropical environment of Africa, Atlas of Dermatological Conditions in Populations of African Ancestry is an essential tool in broadening the scope of care for professionals and residents in dermatology alike.

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