Hiroyasu Endo

Pemphigus Vulgaris Confined to the Gingiva: A Case Report

Pemphigus Vulgaris (PV) is an autoimmune intraepithelial blistering disease involving the skin and mucous membranes. Oral mucosa is frequently affected in patients with PV, and oral lesions may be the first sign of the disease in majority of patients. In some patients, oral lesions may also be followed by skin involvement. Therefore, timely recognition and therapy of oral lesions is critical as it may prevent skin involvement. Early oral lesions of PV are, however, often regarded as difficult to diagnose, since the initial oral lesions may be relatively nonspecific, manifesting as superficial erosions or ulcerations, and rarely presenting with the formation of intact bullae. Lesions may occur anywhere on the oral mucosa including gingiva; however; desquamtive gingivitis is less common with PV than other mucocutaneous conditions such as pemphigoid or lichen planus. This paper describes the case of a patient presenting with a one-year history of painful gingival, who is finally diagnosed as having PV.

Diagnosis and Management of Desquamative Gingivitis

Desquamative gingivitis (DG) is characterized by erythematous gingiva, desquamation and erosion of the gingival epithelium, and blister formation. It is a clinical manifestation common to several diseases (Endo et al., 2008a; Lo Russo et al., 2008; Rees, 2011). It is seen mainly in adults, especially women, although rare cases have been observed in children (Barnett et al., 1981; Cheng et al., 2001; Leao et al., 2008; Lo Russo et al., 2009; Yih et al., 1998). Nisengard and Levine (1995) cited the following as the standard for the clinical appearance of DG: 1) Gingival erythema not resulting from plaque, 2) Gingival desquamation, 3) Other intraoral and sometimes extraoral lesions, and 4) Complaint of sore mouth, particularly after eating spicy foods. Nikolskys sign often shows a positive reaction in patients with DG (Fig. 1). This sign involves the application of a shearing force on normal-appearing gingiva, producing epithelial desquamation. The specificity of Nikolskys sign was higher (96.3%) than the sensitivity (46.7%), indicating that Nikolskys sign is useful in the preliminary diagnosis of oral blistering diseases and may represent a simple clinical tool for oral health practitioners (Mignogna et al., 2008). It was previously thought that DG was caused by a hormone imbalance since it often occurred in middle-aged and older women. However, advanced diagnosis using immunological techniques indicates that most cases of DG are caused by mucocutaneous diseases, the most common ones being lichen planus (LP), mucous membrane pemphigoid (MMP), and pemphigus vulgaris (PV) (Endo et al., 2008a; Leao et al., 2008; Lo Russo et al., 2008; Lo Russo et al., 2009; Nisengard & Rogers, 1987; Rees, 2011; Yih et al., 1998). Contact allergic reactions to various oral hygiene products have also been reported to present as DG (Endo & Rees, 2006, 2007; Endo et al., 2010; Lamey et al., 1990; Rees, 1998, 1999, 2011). Although a definitive diagnosis of the specific disease or disorder causing DG is required to provide proper treatment, it is almost impossible to do so based solely on clinical manifestations. Therefore, histopathological examination and direct immunofluorescence (DIF) testing are often required to establish the final diagnosis. According to a summary of results using DIF, 75.4% of the 174 cases that were clinically diagnosed as DG were caused by mucocutaneous diseases (Nisengard & Rogers, 1987), the most commonly recognized

A stab-and-roll biopsy technique to maintain gingival epithelium for desquamative gingivitis.

BACKGROUND Desquamative gingivitis (DG) is a clinical manifestation common to several diseases. It is known that most cases of DG are caused by mucous membrane pemphigoid (MMP), oral lichen planus (OLP), or pemphigus vulgaris (PV). Early recognition and treatment of these diseases can improve the prognosis, but diagnostic delays are common in patients with DG because obtaining a diagnostic biopsy is technically challenging. A biopsy technique designed to maintain the gingival epithelium for patients with DG was developed. The usefulness of this technique is discussed. METHODS This study is based on a retrospective review of 27 DG cases. A stab-and-roll technique was used to obtain gingival tissue. This technique is designed to reduce lateral forces on the epithelium during the procedure and to thereby prevent the inadvertent removal of the epithelium from the biopsy specimen. A total of 52 biopsies comprising 27 for hematoxylin and eosin (H&E)-stained samples and 25 for direct immunofluorescence (DIF) testing were reviewed. RESULTS Fifty-one of the 52 biopsies (98.1%) maintained the epithelium. Only one biopsy (1.9%) showed that the epithelium was totally absent. Therefore, H&E and DIF features of 51 biopsies were analyzed. Definitive diagnoses of the diseases causing DG included MMP (13 cases), PV (eight cases), and OLP (six cases). CONCLUSIONS A diagnostic biopsy was obtained from the gingiva of patients with DG using the stab-and-roll technique. The gingival epithelium was well maintained, and the relationship with the underlying connective tissue was diagnostic. In the future, this stab-and-roll biopsy technique may facilitate early diagnosis and treatment of diseases causing DG.

Predominant cultivable bacteria and specificity of local antibody responses in juvenile periodontal disease.

若年者における8名の歯周炎の病型を検索した結果, 病変の広がり, 口腔内診査の結果などを加味した臨床的視点から, 限局型と広汎型に分類類した。同一患者の口腔内から“罹患部位と非罹患部位を選択し, 両部位に対してポケット内細菌の同定, 歯肉溝浸出液中の抗体価の測定を行い, 同時に, X線, 研究模型による診査を加え, 形態学的な検索も行った。形態学的診査の結果, 概して上顎第一大臼歯は水平的, 下顎第一大臼歯は垂直的な骨吸収像を示す傾向があり, 限局型では歯槽幅/歯冠径比が大きい場合が多く, また, 咬合関係の不調和がその部位の病変を増悪させている可能性が考えられた。細菌学的には, B. gingivalis, H. actinomycetemcomitans, Capnocytophagaが罹患部位で多く検出された。歯肉溝滲出液中の抗体価は, 罹患部位で高いとは限らず, 非罹患部位で高い場合も認められたため, その部位の病変を抗体価で規定することは困難であると考えられた。