白色海绵状斑痣病例报道及文献复习  

White sponge nevus: a case report and literature review

作　　者：洪鹏宇 高嘉雄 王典日 唐瞻贵[1] HONG Pengyu;GAO Jiaxiong;WANG Dianri;TANG Zhangui(Department of Oral and Maxillofacial Surgery,Xiangya School of Stomatology,Xiangya Stomatological Hospital,Central South University, Changsha 410000,China;State Key Laboratory of Oral Diseases & National Clinical Research Center for Oral Diseases,Department of Oral and Maxillofacial Surgery,West China Hospital of Stomatology,Sichuan University,Chengdu 610041,China)

机构地区：[1]中南大学湘雅口腔医学院,中南大学湘雅口腔医院口腔颌面外科,湖南长沙410000 [2]口腔疾病研究国家重点实验室国家口腔疾病临床医学研究中心四川大学华西口腔医院口腔外科,四川成都610041

出　　处：《口腔疾病防治》2019年第7期464-467,共4页Journal of Prevention and Treatment for Stomatological Diseases

基　　金：国家自然科学基金项目(30872895,81671003);湖南省科技计划项目(2017SK4003)

摘　　要：目的探讨白色海绵状斑痣的病因、临床表现、诊断及治疗方法,为临床诊疗提供参考。方法对1例发生于口腔的白色海绵状斑痣病例的临床资料及相关文献进行回顾性分析。结果白色海绵状斑痣是一种罕见的常染色体显性遗传病,可有家族病史,患者的角蛋白基因K4(Keratin4)与K13(Keratin13)突变被认为是主要病因。该病多始发于儿童及青少年,在成年后趋于稳定,表现为黏膜上广泛白色水波样褶皱,质地柔软,以双侧颊黏膜好发,病理检查通常显示病变部上皮细胞角化过度,棘层细胞内水肿与空泡性变,而基底层细胞大致正常。在临床上需与口腔白斑病、口腔扁平苔藓、口腔念珠菌病等疾病相鉴别。目前尚无特异性治疗方法,常以局部涂抹维A酸,应用漱口水保持口腔卫生清洁为主,患者若无自觉症状也可不予治疗。该病预后良好,无恶变倾向。结论白色海绵状斑痣因较为罕见,易被临床医生忽视,诊断主要依靠病史询问、临床表现和病理检查,今后的研究方向应致力于寻找更为有效的治疗手段。Objective To explore the etiology, clinical manifestation, diagnosis and treatment of white spongy spot nevus, so as to provide reference for clinical diagnosis and treatment. Methods The clinical data and related literature of a case of white cavernous nevus in oral cavity were retrospectively analyzed. Results White spongy nevus is a rare autosomal dominant hereditary disease with a family history. The mutations of keratin gene K4 and K13 in patients with white spongy nevus are considered to be the main causes. The disease usually starts in children and adolescents and tends to be stable in adulthood. It is characterized by extensive white water.wave folds on the mucosa, soft texture, and affects the bilateral buccal mucosa. Pathological examination usually shows excessive keratosis of epithelial cells, edema and vacuolation in spinous cells, while basal cells are generally normal. In clinic, it should be differentiated from oral leukoplakia, oral lichen planus and oral candidiasis. At present, there is no specific treatment method. Retinoic acid is of. ten applied locally and gargle is used to keep oral hygiene and cleanliness. Patients can not be treated without conscious symptoms. The prognosis of the disease is good and there is no tendency of malignancy. Conclusion White spongy nevus is very rare and easily missed by clinicians. Diagnosis mainly depends on medical history, clinical manifestations and pathological examination. Future research directions should be devoted to finding more effective treatment.

关 键 词：白色海绵状斑痣 口腔黏膜 遗传病 口腔白斑 口腔扁平苔藓 基因突变 角蛋白 

分 类 号：R78[医药卫生—口腔医学]