Chapter 16 - Bullous Diseases

Abstract

Immunobullous diseases, while rare, may present severe medical and therapeutic challenges to the treating clinician. These entities include pemphigus and its variants, bullous pemphigoid and its variants, epidermolysis bullosa acquisita, linear IgA bullous dermatosis, and dermatitis herpetiformis. Classification and treatment require an understanding of the basic biology of keratinocyte to keratinocyte and keratinocyte to extracellular matrix interactions as well as the disease-specific autoantibodies that interfere with these relationships. Standard histology in combination with direct and indirect immunofluorescence testing provides the cornerstone for diagnosis. Significant strides have been made within the last decade in the development of non-steroidal medications with the introduction of IVIg and biologic therapies such as rituximab. Improved therapeutics have resulted in decreased mortality and morbidity from the disease states themselves as well as from treatment-induced complications.