[PDF][PDF] Pemphigus and pemphigoid as paradigms of organ-specific, autoantibody-mediated diseases.

JR Stanley - The Journal of clinical investigation, 1989 - Am Soc Clin Investig
JR Stanley
The Journal of clinical investigation, 1989Am Soc Clin Investig
The blistering skin diseases pemphigus and pemphigoid can be considered paradigms of
antibody-mediated, organ-specific autoimmune diseases. These diseases not only
demonstrate mechanisms whereby autoantibodies can mediate tissue damage, but are also
examples ofhow autoantibodies from patients can be used as tools to further our
understandingofthe molecular structure of normal tissue. In this Perspectives article, I will
briefly describe the clinical, histologic, and immunopatho-logic features of these diseases. I …
The blistering skin diseases pemphigus and pemphigoid can be considered paradigms of antibody-mediated, organ-specific autoimmune diseases. These diseases not only demonstrate mechanisms whereby autoantibodies can mediate tissue damage, but are also examples ofhow autoantibodies from patients can be used as tools to further our understandingofthe molecular structure of normal tissue. In this Perspectives article, I will briefly describe the clinical, histologic, and immunopatho-logic features of these diseases. I will then discuss in more detail the newer data regarding the pathophysiology of these diseases, as well as the molecules defined by the autoantibodies from these patients.
Clinical and histologic features The clinical and histologic aspects of pemphigus and pemphi-goid are well established (Table I)(1). There are two major types of pemphigus, called pemphigus vulgaris (PV)'and pemphigus foliaceus (PF). Patients with PV almost always develop, and often present with, mucous membrane erosions. Skin lesions, which are seen as flaccid blisters or erosions, tend to gradually enlarge at the edges. PV, ifleft untreated, is almost always fatal. The histology ofa PV lesion shows that the blister (or erosion) forms because of separation of epidermal cells from each other (a process called acantholysis) just above the basal cell layer. In contrast to PV patients, PF patients rarely have mucous membrane lesions. They usually present with scaly and crusted skin lesions, which, unlike PV lesions, donot tend to form extensive and enlarging erosions, but are fixed and well demarcated. Fogo selvagem (also called Brazilian PF) is a form of PF that is endemic in ruralareas of Brazil and often affects children and young adults (2). The histology ofa PF lesion demonstrates blister formation dueto acantholysis in the superficial epidermis at the granular layer. With these histologic findings in mind, both major types of pemphigus, PV and PF, can be thought of as diseases of epithelial cell adhesion, with the blister or erosion a result ofloss ofadhesion at different levels within the stratified squamous epithelium of skin or mucous membrane.
The Journal of Clinical Investigation