Nutrition and Oral Medicine (Nutrition and Health)

248 Part IV / Select Diseases and Conditionsacterized by auto-antibodies directed against desmosome-associated protein antigens(desmoglein 3 in 100% of cases, desmoglein 1 in 50% of cases) found in epithelial andepidermal intercellular substance (10). Because the desmosome is the primary attachmentmechanism between keratinocytes, the inflammatory destruction of that attachmentleads epithelial separation, formation of characteristic fluid-filled bullae, and subsequentulceration. When the epithelial attachment is compromised or destroyed, even minormucosal trauma may result in epithelial separation (acantholysis) and bullae formation.The bullae quickly rupture, leaving a relatively nonspecific, shallow ulceration with anirregular border.3.2.2. CLINICAL FEATURES3.2.2.1. General FeaturesThe typical presentation of PV is multiple, chronic, shallow mucocutaneous ulcerations,preceded by bullae, occurring most often in patients over age 40. The lesions donot heal without treatment and often develop following minor trauma (referred to as theNikolsky sign). Althogh the oral presentation of PV is by far the most common first siteof lesions, and the exclusive site for approx 25% of patients, it is not well recognized inthe mouth, and, unfortunately, the oral presentation is often associated with significantdiagnostic delays (11,12).3.2.2.2. Oral FeaturesMultiple, shallow, chronic oral mucosal ulcerations are the typical presentation of PV.Generalized desquamative gingivitis is another common presentation of oral PV, appearingas generalized gingival erosion and erythema. Other diseases to be considered in thedifferential diagnosis include benign mucous membrane pemphigoid and erosive lichenplanus; several other disorders may be considered but are exceptionally rare. The oralcomponent of PV is the presenting sign in 80% of patients with PV, and the exclusive sitein 24% of patients (11,12). Despite this predominant oral presentation, the oral presentationis not well recognized and is associated with considerable diagnostic delays (11,12).Oral complications of PV include painful gingival and mucosal lesions that can preventadequate oral hygiene and intake of food, resulting in risks for significant dental andnutritional complications, and complications related to immunosuppressant medicationsused in the treatment of PV (see relevant sections below).3.2.3. DIAGNOSISA perilesional biopsy must be performed and submitted for both routine (hematoxylinand eosin) and direct immunofluorescent (immunoglobulin G [IgG]) stains (8–10).Additionally, circulating antibodies (IgG) are detectable in 80 to 90% of patients withPV; the titer is generally correlated with the level of clinical disease (13) and is a usefulmeasure of treatment effectiveness and disease activity.3.2.4. TREATMENTPV is a serious, chronic, incurable disease that can lead to death. The goal of treatmentis elimination of lesions by reduction in auto-antibody production using systemic therapy;local treatment is of limited value with the exception of topical or injection steroidmedication to manage lesions recalcitrant to therapy. First-line therapy is usually prednisonein moderate to high doses (1–2 mg/kg) (14,15). Doses of prednisone can be