Journal of Otology & RhinologyISSN: 2324-8785

All submissions of the EM system will be redirected to Online Manuscript Submission System. Authors are requested to submit articles directly to Online Manuscript Submission System of respective journal.
bahis siteleri bahis siteleri bahis siteleri casino siteleri

Case Report, J Otol Rhinol Vol: 4 Issue: 6

Plasma Cell Mucositis Affecting Oral Mucosa: Report of 11 Cases and Review of Literature

BSMS Siriwardena1*, Jayasinghe RD2 and Tilakaratne WM1
1Department of Oral Pathology, Faculty of Dental Sciences, University of Peradeniya, Sri Lanka
2Department of Oral Medicine and Periodontology, Faculty of Dental Sciences, University of Peradeniya, Sri Lanka
Corresponding author : BSMS Siriwardena BDS, MPhil, PhD, MD
Department of Oral Pathology, Faculty of Dental Sciences, University of Peradeniya, Sri Lanka
Tel: +94812397536
E-mail: [email protected]
Received: October 13, 2015 Accepted: November 07, 2015 Published: November 10, 2015
Citation: Siriwardena BSMS, Jayasinghe RD, Tilakaratne WM (2015) Plasma Cell Mucositis Affecting Oral Mucosa: Report of 11 Cases and Review of Literature. J Otol Rhinol 4:6. doi:10.4172/2324-8785.1000259

Abstract

Plasma cell mucositis is a rare idiopathic inflammatory condition showing benign plasma cell proliferation with erythematous mucosa and varying surface appearances affecting oral, upper respiratory and genital mucosae. Final diagnosis should be achieved by clinicopathological correlation following exclusion of other similarly presenting diseases. Different treatment modalities such as steroids, calcinurin inhibitors, cryotherapy, laser and surgical excision have been used but with varying response. Therefore Plasma cell mucositis presents a challenge in both diagnosis and treatment. Here, we report eleven new cases of plasma cell mucositis and discuss their histological variations and management.

Keywords: Plasma cell mucositis; Oral mucosa; Mucositis; Ulcer

Keywords

Plasma cell mucositis; Oral mucosa; Mucositis; Ulcer

Introduction

Mucositis is a painful inflammation and ulceration of mucous membranes. Plasma cell mucositis [PCM] is a rare variation of mucositis and is considered as an idiopathic inflammatory condition showing a benign plasma cell proliferation [1]. PCM affects oral, upper respiratory and genital mucosae. It encompasses a clinicopathological combination of an intensely erythematous mucosa with papillomatous, cobblestone, nodular or velvety appearance and a polyclonal plasma cell infiltrate in the superficial lamina propria [2,3].
This was first described as an entity affecting glans penis and was named as “balanitis plasma cellularis” by Zoon in 1952. Subsequently similar lesions affecting upper respiratory tract, oral mucosa and external female genitalia were reported. This has over 20 designations describing the site affected or its idiopathic nature; plasma cell chelitis, atypical gingivo stomatitis, plasma cell vulvitis, idiopathic plasmacytosis etc. [3-5]. In 1986 White et al introduced the term “plasma cell orificial mucositis” to include all these lesions [2]. Ferreiro et al. in 1994 [6] introduced the term “mucous membrane plasmacytosis”. The current term “plasma cell mucositis” was proposed by Smith in 1999 [3]. More recently Brix et al. in 2009 have suggested the term “idiopathic lymphoplasmacellular mucositis-dermatitis”, arguing that upper airway is not “orificial” and external female genitalia is not strictly mucosal [5].
Aetiological factors for PCM have not been established but patients tend to have co existing autoimmune or immunological disorders [2,3]. Presenting symptoms of oral PCM include oral pain, burning sensation, dysphagia, sore throat of long duration and hoarseness. Clinical presentation or the histopathology of a plasma cell rich infiltrate is not specific for PCM. Hence, arriving at the final diagnosis is difficult and is reached by clinico pathological correlation following exclusion of similarly presenting diseases [1]. Different treatment modalities with varying success have been used including corticosteroids, calcinurin inhibitors and surgical excision.
Presently we report eleven new cases of PCM presented to oral medicine clinic and discuss their histological variations and management.

Materials and Methods

Eleven patients who presented to the Department of oral medicine, Faculty of dental sciences, University of Peradeniya, Sri Lanka with the chief complaint of oral discomfort and difficulty in swallowing for 3-9 months duration were taken as the cases. All these patients had been diagnosed as PCM by the histopathological investigations. In this study clinical presentation and histological features of all the cases were analyzed and compared with the literature.

Results

There were 11 patients including 4 males and 7 females. The age ranged from 37-70 years with the mean age of 57.5 years. Male to female ratio was 1:1.75. The most common site affected was lower lip followed by tongue. Clinically most of them showed erosions, ulcerations, erythematous areas affecting single or multiple sites (Figure 1, 2 and 3).
Figure 1: Tongue lesion of Case I showing erythematous areas covering with sluff.
Figure 2: Gingival lesion of Case I.
Figure 3: (A) Lip lesion of case Case XI; (B) Buccal mucosal tags like appearance.
Incisional biopsies were taken from all the cases and sent for histopathological investigations and confirmed as plasma cell mucositis. Histologically the predominant feature was a dense infiltration of plasma cells in the upper corium extending to deeper muscles and minor salivary tissue (Figure 4 and 5).
Figure 4: Histopathologically ulcerated lesion with dense infiltrate.
Figure 5: Higher magnification of deeper corium showing dense plasma cells.
Interestingly, majority of the cases showed histological features of lichenoid reaction. This may partly be due to indegenous topical applications that they received before the biopsy. Clinical and histopathological data were given in Table 1. These patients were treated with corticosteroids (0.05% Clobetasol) and antifungals (0.01% Tacrolimus with Miconazole 2% cream) for one month and remarkable improvement was observed. The patients were followed up regularly and steroid therapy was tailed off gradually (Figure 6).
Table 1: Clinical and histological data of PCM.
Figure 6: Healed tongue following therapy.
Plasma cell mucositis affecting the upper aerodigestive tract is a rare condition with less than 50 cases reported. Hence, no extensive epidemiological data are available. Few small reviews have been presented by Ferreiro et al., Smith et al. and Solomon et al. With each subsequent review, new cases have been added to the previously reported cases (Table 2). Age range, mean age and gender distribution varies among these reviews. In the largest review available by Solomom et al. a wider age range of 27-84 years with a mean age of 56.6 years and a male predilection has been reported. However, in the present series female predominance was observed with the male to female ratio of 1:1.75. In the present series also age range is wider with the mean of 57.5 years which is compatible with the literature.
Table 2: Age and gender distribution in three reviews of PCM.
Several theories related to the possible aetiology of PCM have been proposed, including traumatic, genetic, hormonal, autoimmune and immunological mechanisms including hypersensitivity reaction [7]. ‘’Plasma cell gingivitis’’ is a different entity, well recognized to be a hypersensitivity reaction to highly flavoured foods, dentifrices or chewing gums [1]. Smith et al proposed the possibility of it being a hypersensitivity reaction against antigens of bacteria colonizing upper aerodigestive tract and genital region [3]. However, up to date none of these has been proven Therefore it is considered to be an idiopathic inflammatory condition to an unknown exogenous agent.
More significantly, an associated synchronous or metachronous autoimmune or immunologically mediated disease has been present in most cases. A range of possible diseases such as Sjogren’s syndrome, seronegative arthritis, autoimmune hepatitis and diabetes mellitus have been associated with PCM, though no single condition is constantly found [1-3]. Clinical features typically present in oral PCM are intensely erythematous mucosa described as bright red in colour and with varying surface characteristics such as papillomatous, cobblestone, nodular, or velvety appearances. If gingiva is affected, the attached gingiva may appear erythematous with swelling and the lips may show chelitis with papillary hyperplasia [1].
Histologically, Ferreiro et al. described lesions showing psoriasiform epithelial hyperplasia and spongiosis [4,6]. Erosions and atrophy with vacuolar alteration as well as lozenger shaped keratinocytes were also described in Brix et al.’s review of histological features using 27 cases [5]. More characteristically, in the superficial lamina propria a dense cellular infiltrate composed largely of mature plasma cells. A few neutrophils, lymphocytes and Russell bodies were occasionally noted. In the cases presented here about 64% showed features of lichenoid reaction such as presence of apoptic bodies, focal areas of basal cell destruction other than dense infiltrate of mature plasma cells. Immunohistochemistry revealed mixed population of kappa and lambda light chains positivity confirming the polyclonal nature of the plasma cells [5].
The clinical presentation or histopathological features of plasma cell rich infiltrates are not specific to PCM. Depending on the clinical presentation and histology, differential diagnosis will include mucocutaneos diseases (erosive or atrophic lichen planus, lichenoid reaction and discoid lupus erythematosus), vesciculobullous diseases (benign mucous membrane pemphigoid, pemphigus) mucosal infections (atrophic candidiasis and syphilis), allergic or contact hypersensitivity reactions, granulomatous lesions (sarcoidosis, chelitis granulomatosa, and Wegener’s granulomatosis), neoplastic plasma cell lesions (Primary cutaneous plasmacytoma, extra medullary plasmacytoma) and erythroplakia [1,3,5].
Final diagnosis is only reached by clinicopathological correlation following exclusion of other similarly presenting diseases. This difficulty in diagnosis is reflected by the fact that most cases are described as requiring multiple biopsies and having many other investigations [1]. This was the situation in the present series as well, and majority of them have been seen by several clinicians and had previous biopsies.
Different types of therapy for PCM have been used with varying success but most of these are aimed to give symptomatic relief [1]. The treatment modalities include immune modulation by corticosteroids [topical, intra lesion, systemic or a combination] or calcinurin inhibitors (Tacrolimus, Ciclosporine) and surgical excision. In addition, anti fungal therapy (topical and oral Nystatin, oral griseofulvin) and topical antibiotics (2% fucidic acid) have also been used. For lesions affecting or progressing to affect larynx or pharynx, systemic chemotherapy, low doses of radiation therapy and de-bulking by surgery, CO2 laser, electrocoagulation and cryotherapy have also been reported [4,7-9].
Prognosis of PCM is relatively favourable, though most may not show either progression or regression even with treatment, and a few cases progressing to larynx are reported. Obstruction of airway due to laryngeal PCM has also been mentioned as a major complication [4,6]. Hence, regular review is recommended [3]. However progression to a plasma cell neoplasm has never been reported [1].
In conclusion PCM is a rare idiopathic inflammatory condition showing benign plasma cell proliferation with erythematous mucosa and varying surface appearances affecting oral, upper respiratory and genital mucosae. Final diagnosis should be achieved by clinico pathological correlation following exclusion of other similarly presenting diseases. Different treatment modalities such as steroids, calcinurin inhibitors, cryotherapy, laser and surgical excision have been used but with varying response. Therefore, plasma cell mucositis represents a challenge in both diagnosis and treatment.

References










Track Your Manuscript

Media Partners

Associations