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Pemphigus and Pemphigoid As Paradigms of Organ-Specific, Autoantibody-Mediated Diseases

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Pemphigus and Pemphigoid As Paradigms of Organ-Specific, Autoantibody-Mediated Diseases Pemphigus and pemphigoid as paradigms of organ-specific, autoantibody-mediated diseases. J R Stanley J Clin Invest. 1989;83(5):1443-1448. https://doi.org/10.1172/JCI114036. Research Article Find the latest version: https://jci.me/114036/pdf Perspectives Pemphigus and Pemphigoid as Paradigms of Organ-specific, Autoantibody-mediated Diseases John R. Stanley Dermatology Branch, National Cancer Institute, National Institutes ofHealth, Bethesda, Maryland 20892 The blistering skin diseases pemphigus and pemphigoid can be In contrast to the flaccid blisters, erosions, and crusted considered paradigms of antibody-mediated, organ-specific lesions seen in pemphigus patients, bullous pemphigoid (BP) autoimmune diseases. These diseases not only demonstrate patients classically present with tense blisters on normal-ap- mechanisms whereby autoantibodies can mediate tissue dam- pearing or erythematous skin. Whereas pemphigus lesions are age, but are also examples ofhow autoantibodies from patients due to intraepidermal blisters, histology of a BP lesion indi- can be used as tools to further our understanding of the molec- cates a subepidermal blister with an infiltrate containing eo- ular structure of normal tissue. In this Perspectives article, I sinophils in the superficial dermis and at the epidermal base- will briefly describe the clinical, histologic, and immunopatho- ment membrane zone (BMZ). Clinically and histologically logic features of these diseases. I will then discuss in more similar to BP, herpes gestationis (also called pemphigoid ges- detail the newer data regarding the pathophysiology of these tationis) is a subepidermal, autoantibody-associated blistering diseases, as well as the molecules defined by the autoantibodies disease that occurs during the second or third trimester of from these patients. pregnancy, and spontaneously resolves in the months after delivery. Clinical and histologic features The clinical and histologic aspects of pemphigus and pemphi- Immunopathology goid are well established (Table I) (1). There are two major Over 20 years ago, Beutner and Jordon established that BP and types of pemphigus, called pemphigus vulgaris (PV)' and pemphigus were associated with autoantibodies (3). Both di- pemphigus foliaceus (PF). Patients with PV almost always de- rect immunofluorescence of patients' lesional and perilesional velop, and often present with, mucous membrane erosions. skin and indirect immunofluorescence with patients' sera on Skin lesions, which are seen as flaccid blisters or erosions, tend normal skin substrates, have demonstrated antibody binding to gradually enlarge at the edges. PV, if left untreated, is almost to the same areas of skin that are involved with pathologic always fatal. The histology of a PV lesion shows that the blister change in these diseases (Table I). Thus, BP patients have in (or erosion) forms because of separation of epidermal cells vivo bound and circulating antibodies against the epidermal from each other (a process called acantholysis) just above the BMZ and pemphigus patients have antibodies against the cell basal cell layer. In contrast to PV patients, PF patients rarely surface of epidermal cells. Fogo selvagem patients display the have mucous membrane lesions. They usually present with same immunopathology as other pemphigus patients (4). scaly and crusted skin lesions, which, unlike PV lesions, do not Direct immunofluorescence of perilesional skin from BP tend to form extensive and enlarging erosions, but are fixed patients has also demonstrated C3 at the BMZ in virtually all and well demarcated. Fogo selvagem (also called Brazilian PF) cases. In addition, other elements of the classical and alterna- is a form of PF that is endemic in rural areas of Brazil and tive pathway of complement activation may be present, and often affects children and young adults (2). The histology of a the anti-BMZ antibody is capable of complement fixation by PF lesion demonstrates blister formation due to acantholysis the classical pathway (5). Similarly, in virtually all herpes ges- in the superficial epidermis at the granular layer. With these tationis patients, C3 (and sometimes IgG) is present at the histologic findings in mind, both major types of pemphigus, epidermal BMZ, and the patients have a circulating IgG that is PV and PF, can be thought of as diseases of epithelial cell capable of binding the BMZ of normal skin and fixing com- adhesion, with the blister or erosion a result of loss of adhesion plement (6, 7). at different levels within the stratified squamous epithelium of C3 usually is not present in perilesional skin of pemphigus skin or mucous membrane. patients, but may be present in lesional skin (5). Recent in vitro studies have shown the ability of both PV and PF sera, including fogo selvagem sera, to fix complement by the classi- Address reprint requests to Dr. John R. Stanley, National Cancer cal pathway (8-10). Institute, Dermatology Branch, Building 10, Room 12N238, National An unexplained feature of the direct and indirect immuno- Institutes of Health, Bethesda, MD 20892. fluorescence of pemphigus antibodies is the pattern of binding Receivedfor publication 29 November 1988 and in revisedform 20 PF and PV lesions show blister December 1988. within the epidermis. Although formation at different levels of the epidermis, in most cases the of PF and PV is with 1. Abbreviations used in this paper: BMZ, basement membrane zone; pattern autoantibody binding identical, BP, bullous pemphigoid; PF, pemphigus foliaceus; PV, pemphigus binding on the surface of cells throughout the epidermis (1 1). vulgaris. In a few unusual cases, however, PF antibodies that bind only the superficial epidermis have been reported (12, 13). In gen- The Journal of Clinical Investigation, Inc. eral, though, PF and PV cannot be distinguished on the basis Volume 83, May 1989, 1443-1448 of their direct or indirect immunofluorescence pattern. Pemphigus and Pemphigoid 1443 Table L Clinical, Histologic, Immunopathologic, and Immunochemical Characteristics ofPemphigus and Pemphigoid Pemphigus vulgaris Pemphigus foliaceus Builous pemphigoid Clinical Mucous membrane and skin Scaly, crusted lesions of skin Tense blisters on normal and erosions, flaccid blisters erythematous skin Histologic Loss of adhesion with blister in Loss of adhesion with blister in Subepidermal blister lower epidermis upper epidermis Direct immunofluorescence In vivo bound IgG on cell Same as in pemphigus vulgaris In vivo bound C3 and IgG at surface of keratinocytes epidermal basement membrane Indirect immunofluorescence Serum IgG binds cell surface Same as in pemphigus vulgaris Serum IgG binds normal of keratinocytes throughout epidermal basement normal epidermis membrane Immunochemical 130-kD antigen disulfide Desmoglein, 160-kD 230-kD protein of characterization ofantigens linked to 85-kD polypeptide desmosomal glycoprotein hemidesmosomes Pathogenicity ofautoantibodies studies clearly demonstrate that complement is not required There is excellent evidence that pemphigus and BP autoanti- for blister formation in pemphigus, but the fixation of com- bodies mediate disease. The mechanisms whereby these auto- plement by pemphigus IgG might amplify the resulting antibodies are thought to cause blister formation provide ex- acantholysis. cellent examples of different mechanisms whereby autoanti- Finally, in light of the discussion below of the binding of bodies can mediate disease. pemphigus antibodies to desmosomal components, it must be Initial evidence that pemphigus antibodies cause blister considered that these autoantibodies might interfere directly formation came from studies of skin in organ culture. Both PV with cell adhesion. and PF IgG, without complement, are capable of inducing Although the data are not as definitive as those shown with acantholysis in this organ culture system, at the same level in pemphigus antibodies, BP autoantibodies are probably also the epidermis as in actual disease (14, 15). The pathogenicity pathogenic, not directly but through activation of complement ofboth PV and PF antibodies has been confirmed in an in vivo and recruitment of inflammatory cells. In vivo studies indicate model, in which pemphigus IgG is passively transferred to that BP IgG injected into rabbit corneas causes fixation of IgG neonatal mice that subsequently develop blisters and erosions, and C3 at the corneal BMZ, resulting in inflammation and with acantholysis at the appropriate level of the epidermis subepithelial blister formation (27). Other in vivo studies are (16, 17). not as clear. One study indicates that BP IgG injected in guinea The mechanism whereby these pemphigus antibodies in- pig skin causes subepidermal blister formation, but only in the duce acantholysis is an area of active investigation. Organ and presence of an intact alternative complement pathway (28). cell culture studies have suggested that binding of pemphigus However, another study could not reproduce these findings of autoantibodies to the keratinocyte cell surface causes release of blister formation in guinea pig skin with BP antibodies (29). a protease, which, in turn, causes acantholysis (18-20). The Some of the more convincing studies of the pathogenicity of identity of this proteolytic enzyme is in dispute. Some studies BP IgG, and the pathophysiology of blister formation, come suggest that it is plasminogen activator that, once released by from in vitro studies in which BP IgG and a source of comple- keratinocytes, activates plasminogen to plasmin,
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