Autoimmune Blistering Diseases

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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.

In the first volume of this two part issue devoted to Autoimmune Blistering Diseases (AIBD), diagnosis and clinical features of these diseases are covered by an internationally recognized group of authors. Topics include Pemphigus Vulgaris, Pemphigus Foliaceus, Linear IgA Bullous Disease, and Hair and Nail Loss in AIBD.

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.

Part II of Dr. Murrell’s issue devoted to Autoimmune Blistering Diseases focuses on the management of these conditions along with drug treatment, complications, and the treatment of AIBD around the world. Articles include corticosteroid use in AIBD, AIBD management in pregnancy, prevention of opportunistic infections, and therapeutic approaches to patients with mucous membrane pemphigoid, as well as AIBD management in France, Iran, the United States and other countries.

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.