Pemphigus Vulgaris

PV is a chronic autoimmune mucocutaneous disease in which autoantibodies directed at structural proteins of the desmosome destroy the inter-epithelial cell attachments, producing acantholysis and subsequent suprabasal, intraepithelial bullae (30,31). The targets of the autoantibodies are the transmembrane glycoproteins desmoglein 1 and 3. PV is one member of a group of diseases characterized by autoimmune attack on the desmosomal attachment apparatus. Other members of the group include pemphigus foliaceus, vegetans, and erythematosus, which are less severe than PV. In the early stages, PV may be confined to the oral mucosa. In fact, oral mucosal lesions may be the presenting sign of the disease. The typical patient profile is an older adult. There is a higher incidence in certain populations sharing specific histocompatibility antigens, such as Ashkenazi Jews. Drug-induced pemphigus has been reported secondary to a number of drugs, including penicillamine, phenobarbital, and rifampin. Paraneoplastic pemphigus also occurs in conjunction with non-Hodgkin's lymphoma and chronic lymphocytic leukemia

FIGURE 36 Pemphigus vulgaris. This 31-year-old female complained of a six-month history of oral mucosal discomfort. Note the posterior soft palate location of the collapsed, fragile intraepithelial bullae. Source: Courtesy of Bruce Barker, DDS.

FIGURE 36 Pemphigus vulgaris. This 31-year-old female complained of a six-month history of oral mucosal discomfort. Note the posterior soft palate location of the collapsed, fragile intraepithelial bullae. Source: Courtesy of Bruce Barker, DDS.

FIGURE 37 Pemphigus vulgaris with desquamative gingivitis. This adult female had a six-month history of desquamative lesions of the gingival and soft palate. In contrast to collapsed bullae of mucous membrane pemphigoid, which frequently billow up in response to a tangential stream of low-pressure air (Fig. 33), the bullae of pemphigus vulgaris are much more fragile and the epithelium simply piles up and desquamates in response to the air stream. Source: Courtesy of Charles Dunlap, DDS.

and a number of other neoplasms. PV most frequently involves the more posterior areas of the oral cavity (Fig. 35) such as the soft palate (Fig. 36).

Differential Diagnosis

The intraepithelial location of the bullae in PV makes them fragile and short-lived. They quickly rupture to form secondary eroded lesions. Acantholytic keratinocytes, termed "Tzanck cells," may be seen in exfoliative cytologic smears of bullous lesions. PV, erosive lichen planus, and MMP may have similar clinical features and all may present with desquamative gingivitis. They may be differentiated by DIF testing. In PV, DIF exhibits reteform, net-like deposit of immunoreactants (IgG) outlining the periphery of the epithelial cells in the spinous layer. In contrast to the collapsed bullae of MMP, which frequently billow up in response to a stream of low-pressure air, the bullae of PV are much more fragile and the epithelium simply piles up and desquamates in response to the air stream (Fig. 37).

Treatment, Complications, and Prognosis

Management of PV requires systemic corticosteroids, usually in combination with steroid-sparing immunosuppressive agents such as azathioprine. In addition to the clinical response, the effectiveness of therapy may be monitored with measurement of circulating

FIGURE 38 Erythroplakia. Toluidine Blue vital staining of the lesion shown in Figure 23. The areas of blue uptake represent areas of DNA concentration and were used to guide the selection of an appropriate biopsy site.

autoantibody titers. Prior to the advent of immunosuppressive therapy, PV was associated with a dismal prognosis with mortality as high as 80% secondary to infection and electrolyte disturbances. Although the mortality rate is currently much lower, the major causes of death in PV are now related to complications of the treatment (long-term corticosteroids).

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