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.
Hundred years ago, Paul Ehrlich speculated whether an individual is able to produce toxic autoantibodies and about the implications of such antibodies for disease. The contention that an alteration of the body fluids causes disease followed the traditional teachings of Hipppocrates and Galen that disease results from dysfunction of the four humors. However, Ehrlich introduced the novel concept of antigen specificity that was based on his side chain theory of antibody formation: (1) antibodies are naturally occuring substances that serve as receptors on the cell surface; (2) the specificity of antibody for antigen is determined by a unique stereochemical configuration of atoms that permits the antibody to bind tightly and chemically to its appropriate antigen; (3) the number of different combining sites structures available is so great that each one differs from the others, with little or no cross reactivity among them; (4) and in order to induce active antibody formation, it is only necessary that appropriate receptors be present on the cells for antigen to interact with them and so stimulate their overproduction and liberation into the blood. According to this description by Paul Ehrlich, the antibody appeared to be a polymorphous cytoplasmic agent with a unique feature – a highly organized combining site (the haptophore group) that determined its unique antigen specificity.
It was Bordet who showed that anti-erythrocyte antibodies were capable of mediating immune hemolysis giving rise to the idea that self-produced hemolytic antibodies might assist in destroying autologous erythrocytes. This and similar findings including the description of cytotoxic antibodies against a variety of other cell types prompted Ehrlich to say: “… the organism possesses certain contrivances by means of which the immunity reaction, so easily produced by all kinds of cells, is prevented from acting against the organism’s own elements and so giving rise to autotoxins…so that we might be justified in speaking of a ‘horror autotoxicus’ of the organism. These contrivances are naturally of the highest importance for the individual” (P. Ehrlich and J. Morgenroth, Berlin. Klin. Wochenschr., 1901)
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