Based on recent advances in the understanding of the immunological pathogenesis of many chronic inflammatory disorders there is increasing evidence that several of them are characterized and potentially mediated by autoimmune phenomena. Classical examples are rheumatoid arthritis, myasthenia gravis, pemphigus vulgaris, lupus erythematosus and multiple sclerosis. Others, such as psoriasis vulgaris, some less well-characterized collagen vascular disorders, vasculitides and a subtype of chronic urticaria have a more or less pronounced autoimmune background that has to be considered in the overall management of these disorders. A significant portion of autoimmune diseases precipitate primarily or secondarily at the skin. Understanding the cutaneous symptoms may be therefore crucial for the diagnosis, classification and therapeutic management of organ-specific and systemic disorders that require special attention by the physician.
This book is set out to present the most recent scientific and clinically relevant state-of-the-art-knowledge on the broad spectrum of autoimmune disorders affecting the skin. It is meant to provide the most recent information on these disorders for clinicians as well as practicioners in dermatology, medicine, rheumatology, ENT, pediatrics, ophthalmology, orthopedics etc and for basic scientists interested in human autoimmunity. Each book chapter dealing with a distinct cutaneous autoimmmune disorder consists of an introduction focusing on the state of knowledge regarding pathogenesis and epidemiology followed by a practical guide how to identify and handle the particular disor-der(s). Special attention is paid to genuine cutaneous autoimmune disorders such as autoimmune bullous skin disorders including pemphigus, pemphigoid and epidermolysis bullosa acquisita. These disorders can be considered as paradigms of organ-specific autoimmune disorders because autoantigens and autoantibody-mediated pathogenesis are well-characterized.
Major progress has been made in the diagnosis and classification of collagen vascular disorders such as systemic sclerosis, lupus erythematosus, dermato-myositis and overlap syndromes. These advances have provided the basis for more specific therapeutic interventions. Recent pathogenetic findings in psoriasis, lichen planus and chronic urticaria have led to novel therapeutic concepts that will replace the "classical" symptomatic treatments that have been established for decades. One striking example is the therapeutic effect of biologics in severe psoriasis vulgaris and psoriatic arthritis and the modulatory effect of high dose immunoglobulins in dermatomyositis and severe vasculitides. In addition to the book chapters on distinct clinical cutaneous disorders, the introductory chapter explains basic immunological principles leading to autoimmunity and the final chapter gives an overview of the mode of action of novel immunomodulatory drugs. The present book which is edited by my co-worker Dr. Michael Hertl is set out to combine major scientific advances in the understanding of autoimmunity with the clinical presentation and management of these disorders. I am convinced that the book constitutes a very successful effort to provide a handbook for those who are scientifically or clinically interested in autoimmune disorders of the skin. I wish the editor and the authors success with this endeavor.
Erlangen, July 2001
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