Prosthodontic Rehabilitation of an Oral Pemphigus Vulgaris Patient

Phemphigus is a serious autoimmune disease characterized by acantholysis and subsequent blistering of the skin and oral mucosa, mediated by autoantibodies directed against intercellular antigens desmoglein-3 and desmoglein-1.1-3 There are three immunologic forms of pemphigus; pemphigus foliaceus, pemphigus vulgaris (PV) and paraneoplastic pemphigus.4 PV accounts for 70 of the cases.5 The disease is rare with potentially fatal consequences and has a reported incidence of 0.1 to 0.5 cases per 100,000 individuals worldwide per year.6,7 It is seen worldwide with a predilection of Mediterranean people and Ashkenazi Jews.5 It is slightly predominant in women and primarily manifests in adults during the fifth and sixth decade of life.7 Oral mucosa is most often the initial site, and in many cases the only site, of disease penetration.6 Any site in the mouth may be involved, but the soft palate, buccal, gingival and lower lip mucosa usually predominate.8,9 The most common clinical presentation of PV is multiple, chronic ulcerations whose color is red, white or a mixture of both.10 Affected oral tissues are highly friable with a tendency to bleed and sheer when subjected to minor trauma.11 Gentle lateral pressure applied to an area adjacent to the affected site forms a blister resulting in a positive Nikolsky’s sign.12 Gingival involvement may manifest as desquamative gingivitis.13 Patients affected by these chronic, painful ulcers often complain of discomfort with eating spiced or sour foods and performing daily oral functions, including routine oral hygiene.8 The definitive diagnosis of PV cannot be based solely on clinical examination, as several other oral vesiculobullous and ulcerative lesions have a similar appereance such as lichen planus, pemphigoid and erythema multiforme.14,15 An incisional, perilesional biopsy containing intact epithelium is required for a definitive diagnosis.10 Since development of oral lesions may precede skin lesions, it is not common for a patient having this disease to seek care first from a dentist.16 The article reports a severe case of PV who was rehabilitated prosthodontically with full-mouth fixed partial prosthesis.