Case report: Oral pemphigus vulgaris with multiple oral polyps in a young patient

A rare case of oral pemphigus vulgaris is presented, which developed initially in a 9-year old Jordanian male. The disease was not well controlled with immunosuppressive therapy and was complicated by the development of multiple oral polyps approximately one year after the onset of symptoms. These lesions were smooth, painless and located particularly on the upper labial gingiva, the labial and buccal mucosae and the tongue. Disease activity continued for approximately nine years until the patient was referred to an oral medicine specialist. Histological examination of the polyps revealed exuberant granulation tissue. Adjustment of the steroid dosage lead to resolution of the oral symptoms and the polyps reduced in number and in size. This case highlights the occurrence of pemphigus vulgaris in young patients, illustrates a rare complication of persistent ulcero-inflammatory disease and emphasises the importance of specialist referral in the management of oral disease.     

Rituximab for the treatment of corticosteroid – refractory pemphigus vulgaris with oral and skin manifestations

J Oral Pathol Med (2011) 40 : 616–620 Pemphigus vulgaris (PV) is a life‐threatening autoimmune blistering disease affecting the skin and mucosa and is associated with increased morbidity and mortality. Once the diagnosis is established, the main stay of treatment of PV is with systemic corticosteroids to control the disease and then to consolidate the management with other immunosuppressive agents. A small group of patients with severe pemphigus, however, remain relcalcitrant to both steroids and azathioprine, and disease is difficult to control. When considering the treatment of refractory PV, there is increasing evidence for the successful use of the monoclonal anti‐CD20 antibody, rituximab. We report here six cases of patients presenting with oral and skin PV with recalcitrant or rapidly progressive disease treated with a novel dosing regimen of rituximab as a single agent. All patients achieved a complete response to a maximum follow‐up of 34 months.     

Quantification and distribution of lymphocyte subsets and Langerhans cells in normal human oral mucosa and skin

Normal human oral (check) mucosa was studied to discover whether the oral cavity resembles the Mucosal Immune System (MIS) or the Skin Immune System (SIS). Immunophenotypes of lymphocyte subsets and Langerhans cells (LC) with their exact locations in the epithelium and papillary layer of the normal buccal mucosa were determined and compared with data of normal human skin. In a double staining procedure, the distribution of T-lymphocytes in relation to blood and lymph vessels was determined. Immunophenotyping of LC was done with a CD1a monoclonal antibody. In contrast to the skin, T-lymphocytes in buccal mucosa are not primarily perivascular in location. They are more or less randomly distributed on both sides of the basement membrane. The epithelium of the buccal mucosa contains about 37 times as many T-lymphocytes as the epidermis of normal skin. T-cell numbers in the papillary layer are more or less comparable. The CD4/CD8 ratios of about 1/2 in the epithelium of buccal mucosa and 1/4 in the skin indicates preferential presence of the CD8 subset in both sites, but the helper/inducer T-lymphocytes play a much greater role in the epithelium of the buccal mucosa when compared with skin. B-lymphocytes were not found in the epithelium and papillary layer of the buccal mucosa. Thus, immune response associated cells in buccal mucosa do not show the MIS pattern since B cells are absent. It has more in common with SIS but differences are also apparent. In the epithelium of the buccal mucosa the density of LC does not differ significantly from that of the skin, but the papillary layer of the buccal mucosa contains significantly fewer LC than the skin. As in the skin most of the LC of the buccal mucosa are found in the epithelium.     

Clinicopathological features and malignant transformation of oral lichen planus: A 12-years retrospective study

Abstract

Objective. Oral lichen planus (OLP) is known to be associated with the risk of developing oral squamous cell carcinoma (OSCC). The objective of this study was to investigate the clinicopathological features of OLP and the prevalence of malignant transformation in this setting. Materials and methods. This retrospective study was carried out on 204 medical records of patients with histologically proven OLP who received long-term follow-up (range 6 months–12 years). Data were entered in an informatic database. The statistical analysis, when needed, was performed with the chi-squared test for significance (p < 0.05). Results. At the moment of the diagnosis, out of 204 patients (163 female and 41 male; mean age 54.5 years), 107 patients (52.45%) suffered from systemic chronic diseases, in particular 46 (22.5%) from hepatitis C. Clinically, the reticular form of OLP was the predominant one and most patients had multiple oral sites of involvement. Fourteen patients showed extra-oral lesions. A percentage of malignant transformation less than 1% was found. In fact, two patients (0.98%) underwent a malignant transformation at a site previously diagnosed as OLP. Conclusions. At present, OLP is accepted as being a potential malignant disorder, therefore lifelong follow-up is recommended.     

口腔黏膜黑色素瘤51例临床分析

目的 探讨口腔黏膜黑色素瘤（oral mucosal melanoma,OMM）的治疗和预后。方法对51例OMM患者的临床资料进行回顾性分析。结果51例中病理证实淋巴结转移31例,其中29例限于Ⅰb～Ⅲ区;本组3、5年生存率分别为35％和21％。切取活检与切除活检、有无色素沉着未显著影响预后;接受手术+生物治疗或生物化疗者生存率显著高于其他治疗模式(P =0.003)。结论OMM颈淋巴结转移主要限于Ⅰb～Ⅲ区;切取活检和色素沉着未显著影响预后;该病预后差,手术+生物治疗或生物化疗有利于改善预后。     

体外连续培养人口腔粘膜角化细胞

目的 建立一个稳定的人正常口腔粘膜角化细胞体外培养体系。方法 取正常口腔粘膜,经Ｄｉｓｐａｓｅ及胰蛋白酶消化获取细胞,采用无血清培养液进行原代及传代培养,并进行形态学观察和角蛋白免疫组化染色等一系列细胞定性研究。结果 获取的细胞为单一上皮细胞;细胞可连续传４～５代,成活３０～５０ｄ;细胞呈铺路石状,为上皮细胞的典型形态;电镜下见细胞具有丰富的张力丝和桥粒等特征性超微结构;角蛋白免疫组化染色阳性。结     

口腔黏膜良性淋巴组织增生病的免疫组化研究

目的　研究口腔黏膜良性淋巴组织增生病与口腔癌的细胞增殖能力、血管密度和细胞凋亡的变化。方法　采用免疫组化SP法检测 15例黏膜良性淋巴组织增生病、9例黏膜良性淋巴组织增生病伴上皮异常增生、15例口腔癌及 10例正常黏膜组织中Ki 6 7的表达、细胞凋亡及微血管密度。结果　口腔黏膜良性淋巴组织增生病伴异常增生及鳞状细胞癌中Ki 6 7的表达明显高于不伴异常增生的口腔黏膜良性淋巴组织增生病及正常黏膜 (P <0 0 5 ) ,在所有的口腔黏膜良性淋巴组织增生病及鳞状细胞癌中微血管密度均明显高于正常组 (P <0 0 5 )。口腔黏膜良性淋巴组织增生病中细胞凋亡明显高于正常黏膜及口腔癌 (P <0 0 5 )。结论　在伴有上皮异常增生的口腔黏膜良性淋巴组织增生病中Ki 6 7表达及微血管密度均介于正常组织和口腔癌之间 ,凋亡细胞数也明显多于正常组织。研究结果提示 :口腔黏膜良性淋巴组织增生病是一种具有癌变潜能的疾患     

细胞角蛋白CK19在口腔粘膜癌变过程中的变化

目的：探讨口腔粘膜癌变过程中CK19的变化。方法：收集正常口腔粘膜、上皮单纯增生、上皮异常增生和口腔鳞癌活检标本共53例，用免疫组化、电泳和Western杂交方法研究上皮中CK19的表达。结果：CK19表达于有上皮异常增生的复层鳞状上层和口腔鳞癌尤其是低分化鳞癌的癌细胞中。随病变程度加重，CK19表达的阳性率，表达强度和占细胞角蛋白构成比显著增加。结论：CK19表达于粘膜上皮的基底上层可作为口腔癌前病变的辅助标志，CK19表达增加是 口腔粘膜病变过程中的早期事件。     

Clinical features of gingival pemphigus vulgaris

BACKGROUND: Pemphigus vulgaris (PV) is a rare, chronic, intraepithelial bullous disease with a potentially fatal outcome. Oral lesions are a hallmark of PV and occur in almost all cases, and represent the preliminary symptom in more than half of the patients. Gingival lesions are very common and, when solitary, often first recognized by periodontist. METHOD: In the literature, gingival localization of PV are usually described as desquamative gingivitis (DG) and/or as vesiculobullous lesions of the free and attached gingiva; in our experience, early lesions only rarely appears as extensive erythema and erosions. CONCLUSIONS: PV at the onset may frequently appear on gingiva as isolated blisters and/or erosions mainly located to the free gingiva, very little in extension and hardly to recognize as bullous lesions.