BP180NC16a autoantibody positivity may predict low-risk mucous membrane pemphigoid.

OBJECTIVE: The objective of the present study was to explore the clinical characteristics of BP180NC16a autoantibody-positive mucous membrane pemphigoid. SUBJECTS AND METHODS: Data from 22 patients were collected by retrospective chart review. For the detection of BP180NC16a autoantibody, a commercially available enzyme-linked immunosorbent assay or chemiluminescent enzyme immunoassay kit was used. The patients were classified into the following clinical phenotypes: low-risk patients who had lesions only in the oral mucosa or in the oral mucosa and skin and high-risk patients who had lesions in the oral mucosa and any of the following sites: the eyes, upper respiratory tract, or esophagus. RESULTS: Eleven of the 22 patients (50%) were BP180NC16a-positive at the time of diagnosis. All the positive patients were in the low-risk group. There was a significant difference in the incidence of low-risk mucous membrane pemphigoid between BP180NC16a autoantibody-positive and BP180NC16a autoantibody-negative patients (p = 0.004). Patients' age, disease duration, and oral disease activity scores were not significantly different between the two groups. CONCLUSION: BP180NC16a reactivity was associated with the clinical phenotype characterized by only oral mucosal lesions or combined oral mucosal and skin lesions. BP180NC16a autoantibody might be useful as a serum marker to predict low-risk mucous membrane pemphigoid.

High frequency of upper aerodigestive tract manifestations in mucous membrane pemphigoid.

OBJECTIVE: The objective of this study was to evaluate the frequency of upper aerodigestive tract involvement in patients with mucous membrane pemphigoid associated with desquamative gingivitis. SUBJECTS AND METHODS: Data from 25 patients were collected by retrospective chart review. Their upper aerodigestive had been evaluated using a conventional flexible fiberscope. Oral disease activity was quantified on the basis of the Mucous Membrane Pemphigoid Disease Area Index activity score. RESULTS: Lesions of the upper aerodigestive tract were confirmed in nine symptomatic patients (9/25, 36%), of which five (5/25, 20%) had laryngeal involvement. No lesions were seen in the asymptomatic patients on fiberscope examination. There was a statistically significant difference in the symptoms, high oral disease activity score, and linear IgA deposition on direct immunofluorescence between patients with and without upper aerodigestive tract lesions (p = .001, .001, .002, respectively). CONCLUSION: The high frequency of considerable complications highlights the importance of confirming the presence of upper aerodigestive tract involvement in patients with mucous membrane pemphigoid having desquamative gingivitis. Signs including the presence of symptoms, high oral disease activity score, or linear IgA deposition on direct immunofluorescence might indicate a higher risk of upper aerodigestive tract involvement.

Acantholysis may precede elevation of circulating anti-desmoglein 3 antibody levels in pemphigus vulgaris presenting with desquamative gingivitis.

Pemphigus vulgaris (PV) is an autoimmune, blistering disease that affects the mucosa and skin. The current theory favors the concept that anti-desmoglein (Dsg) 3 autoimmunity is the only pathogenic event needed to induce acantholysis. However, a few cases of active PV in the oral cavity had no detectable anti-Dsg 3 antibody. The aim of this study was to evaluate the differences in clinical and laboratory findings, whether or not the anti-Dsg 3 antibodies were present. This study was based on a retrospective review of 10 PV cases. The evaluation of the circulating autoantibody titers to Dsg 3 was conducted by using enzyme-linked immunosorbent assay (ELISA). An index value of 20 or more was used as the cutoff for a positive reaction. Only five of the 10 PV cases had a positive Dsg 3 ELISA. There were no differences in clinical, cytological, histopathological, and direct immunofluorescence findings, whether or not the anti-Dsg 3 antibodies were present. Of the five patients with a negative reaction at the time of diagnosis, the Dsg 3 ELISA became positive in the follow-up period in three cases. In the remaining two cases, the Dsg 3 ELISA was consistently negative for 18 months. Dsg 3 ELISA was negative early in some PV cases. Therefore, PV acantholysis may precede the elevation of circulating anti-Dsg 3 antibody levels. The diagnosis of PV should be considered based on comprehensive clinical, histopathological, and immunofluorescent criteria.

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.

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.

Use of oral exfoliative cytology to diagnose desquamative gingivitis: a pilot study.

OBJECTIVE: To evaluate the usefulness of exfoliative cytology as a diagnostic tool for patients with clinical symptoms of desquamative gingivitis. METHOD AND MATERIALS: Fifteen patients clinically diagnosed with desquamative gingivitis were evaluated. At their first visit, a cytologic smear was performed on the gingival lesion. On their second visit, a gingival biopsy was obtained from the perilesional site. Control smears were taken from 15 patients who had no oral lesions. RESULTS: The cytologic findings showed diffuse or collective Tzanck cells in 3 of the 15 cases. The findings of the other 12 cases were nonspecific inflammatory changes compared to the control. The definitive diagnosis for each case was made according to histopathologic and direct immunofluorescence (DIF) findings. The 3 cases in which Tzanck cells were recognized in the cytologic smear were subsequently diagnosed as pemphigus vulgaris. The cases in which only nonspecific cytologic findings were obtained were later diagnosed by biopsy and DIF as mucous membrane pemphigoid or lichen planus. CONCLUSION: All the cytologic findings of the 15 patients, except for 3 cases in which Tzanck cells were found, were nondiagnostic. Therefore, the use of exfoliative cytology does not appear to be appropriate as a diagnostic tool for patients showing the clinical symptoms of desquamative gingivitis, because this technique adds to the cost and delays the definitive diagnosis. However, using the cytologic technique may occasionally be of some value as a minimally invasive screening tool when pemphigus vulgaris is suspected.

Successful treatment using occlusive steroid therapy in patients with erosive lichen planus: a report on 2 cases.

Gingival involvement in erosive lichen planus (ELP) is classified as nonplaque-induced gingival lesions. Occlusive steroid therapy (OST) using a plastic stent is administered to enhance the effect of corticosteroids by maximizing the contact between the corticosteroids applied on the gingiva and the gingival lesions. Clinical efficacy may be enhanced with OST even when low-potency topical corticosteroids are used. The treatment of 2 cases of gingival ELP with symptoms of intraoral pain and a long-term burning sensation is presented. In both cases, severe erythema was found in the attached gingiva. Plastic stents were constructed to provide OST. The patient was instructed to apply topical corticosteroid (0.1% triamcinolone acetonide) to the inner surface of the stent and wear the stent continuously between meals and at night. The condition of the gingiva dramatically improved by providing OST for 10 weeks (case 1) and 3 weeks (case 2). The improvement in the gingival condition has been controlled for a long period (7 to 10 months) even after OST was completed. The treatment methods shown in this report are encouraging and worthy of further study.

Disease progression from mucosal to mucocutaneous involvement in a patient with desquamative gingivitis associated with pemphigus vulgaris.

BACKGROUND: Pemphigus vulgaris (PV) frequently begins with oral lesions and progresses to skin lesions. A patient is described who developed skin lesions during follow-up and whose only initial symptom was desquamative gingivitis (DG). METHODS: A 31-year-old woman presented with a 2-month history of painful gingiva. The diagnosis of PV was made according to clinical, histopathological, and immunofluorescent criteria. Topical corticosteroid (0.1% triamcinolone acetonide) was provided for the treatment of DG. Evaluation of the circulating autoantibody titers to desmoglein (Dsg)1 and Dsg3 was conducted by enzyme-linked immunosorbent assay (ELISA). RESULTS: The gingival PV lesions went into remission with the use of topical corticosteroid, although the patient experienced occasional recurrent oral lesions that required retreatment. She had regular follow-ups and remained relatively stable for several months. However, relapse and worsening of the oral lesions and the onset of skin lesions occurred after 26 months. Using ELISA, a change in the autoantibody profile corresponding to the transition from mucosal PV to mucocutaneous PV was confirmed. In all ELISA studies conducted throughout the course of the patient's disease, the Dsg3 ELISA was consistently high ranging from 150 to 200. However, the Dsg1 ELISA remained low, ranging from 10 to 30. After 26 months, Dsg3 (index value of 150) and Dsg1 (index value of 114) ELISA levels were elevated, consistent with the transition to mucocutaneous PV. CONCLUSIONS: In cases in which the lesions are limited to the oral cavity, PV sometimes may be managed successfully using only topical corticosteroids. However, it may not be possible to reduce the circulating Dsg autoantibody titers without systemic immunosuppression. The sustained high Dsg3 antibody level may cause "epitope spreading" and induce skin lesions. It may be prudent to determine post-treatment levels of Dsg using ELISA and, in consultation with the physician, recommend the addition of systemic therapy if Dsg3 levels remain elevated.

Cinnamon products as a possible etiologic factor in orofacial granulomatosis.

OBJECTIVES: It has been reported that clinical changes due to hypersensitivity reactions to various foods, preservatives, and oral hygiene products may be consistent with the characteristic signs of orofacial granulomatosis (OFG). The objective of this study was to examine 37 well-documented cases of cinnamon-induced contact stomatitis for clinical and histological features consistent with a diagnosis of OFG. STUDY DESIGN: We reviewed the records of the 37 cases to screen them for the clinical and histopathologic features of OFG. RESULTS: Twelve patients showed clinical characteristics of OFG. The most commonly affected site was the gingiva. Focal non-caseating, epitheloid granulomas were observed in four histologic specimens. Multinucleated giant cells were observed in an additional four cases. CONCLUSIONS: Although OFG may have multiple etiologies, it is clear that, in some instances, a hypersensitivity reaction to cinnamon products can elicit lesions consistent with OFG.

Cinnamon products as a possible etiologic factor in orofacial granulomatosis

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Objectives: It has been reported that clinical changes due to hypersensitivity reactions to various foods, preservatives, and oral hygiene products may be consistent with the characteristic signs of orofacial granulomatosis (OFG). The objective of this study was to examine 37 well-documented cases of cinnamon-induced contact stomatitis for clinical and histological features consistent with a diagnosis of OFG. Study design: We reviewed the records of the 37 cases to screen them for the clinical and histopathologic features of OFG.Results: Twelve patients showed clinical characteristics of OFG. The most commonly affected site was the gingiva. Focal non-caseating, epitheloid granulomas were observed in four histologic specimens. Multinucleated giant cells were observed in an additional four cases.Conclusions: Although OFG may have multiple etiologies, it is clear that, in some instances, a hypersensitivity reaction to cinnamon products can elicit lesions consistent with OFG

Clinical and diagnostic features of mucous membrane pemphigoid.

The aim of this study was to characterize the clinical and diagnostic features of mucous membrane pemphigoid (MMP). Five MMP patients were evaluated, and biopsies were obtained for routine histopathology and direct immunofluorescence. Circulating autoantibodies were verified by indirect immunofluorescence. Circulating immunoglobulin G autoantibody specific for 180 kDa bullous pemphigoid antigen (BP180) was evaluated by enzyme-linked immunosorbent assay (ELISA). All 5 patients complained of erythematous gingiva. Two patients had extraoral lesions involving the nasal mucosa, eyes, and skin. Histopathological confirmation was established for 4 of the patients, while direct immunofluorescence findings were positive in all 5. Although circulating autoantibodies were not identified by indirect immunofluorescence, the BP180 ELISA was positive in 3 cases. Both histopathological and direct immunofluorescence examinations are essential to establish a definitive diagnosis. Identification of circulating BP180 specific autoantibody shows promise.

Clinical features of cinnamon-induced contact stomatitis.

Cinnamon flavoring agents are known to act on the mucosa as irritants or sensitizers. The objective of this study was to report on clinical and histologic features important in the diagnosis of cinnamon-induced contact stomatitis. We examined 37 past cases of contact stomatitis and identified clinical signs and symptoms and possible causes. The most common cause in the 37 cases was toothpaste; other causes were chewing gum and foods. Although various clinical findings were observed, some characteristics were evident. The most commonly affected site was the gingiva, which showed diffuse or generalized erythema and epithelial sloughing. It is important to identify the causative agents of such a disorder by understanding characteristic signs and symptoms and conducting an appropriate inquiry.

Early detection and successful management of oral pemphigus vulgaris: a case report.

BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune disease characterized by acantholysis in the epithelium. PV is often difficult to diagnose in the early stages, since the oral lesions are relatively nonspecific. We report on a case with a favorable outcome due to early diagnosis and effective treatment of oral lesions. METHODS: A 53-year-old Japanese woman presented with a month-long history of painful gingiva. PV was diagnosed using clinical, histopathological, and direct immunofluorescence criteria 1 month after the first visit. Evaluation of the circulating autoantibody titers to desmoglein (Dsg)1 and Dsg3 was conducted by enzyme-linked immunosorbent assay (ELISA). Occlusive steroid therapy (OST) using a topical corticosteroid (0.1% triamcinolone acetonide) was provided for 8 weeks. After OST was completed, a buccal frenectomy was performed to eliminate localized toothbrushing trauma. RESULTS: There were no adverse effects on wound healing after the procedure. No oral PV lesions were detectable at the 9-month reevaluation, and the patient remains in complete remission. The Dsg1 ELISA was negative while the Dsg3 ELISA was indeterminate at the first visit. The Dsg1 ELISA was also negative at reevaluation, but Dsg3 ELISA reactivity had increased 9 months after completion of treatment. CONCLUSION: Large scale clinical trials will be required to validate the clinical benefits of the OST treatment regimen, and further research is necessary to determine the importance of ELISA antibody examination in long-term management of oral PV.