姐妹同患硬斑病合并桥本氏甲状腺炎二例并文献复习

发布时间：2018-07-10 01:30

本文选题：硬斑病 + 硬化性萎缩性苔藓　；参考：《大连医科大学》2015年硕士论文

【摘要】：背景:硬斑病(morphea)又称局限性硬皮病(localized scleroderma,LS),以真皮增厚或硬化伴皮下脂肪缺失为特征的自身免疫性疾病。女性多见,其确切病因尚不明确,可能与外界因素、遗传、自身免疫等有关。硬斑病虽不累及内脏器官,没有雷诺现象和甲襞毛细血管的变化,但是LS也可累及皮下脂肪,筋膜,肌肉和骨骼,影响关节活动甚至致残,发生在头面部时可出现神经系统和眼部并发症。分为5个亚型:局限型硬斑病、线状硬斑病、泛发性硬斑病、全身性硬化性硬斑病和混合性硬斑病。硬斑病和硬化性萎缩性苔癣(Lichen sclerosus et atrophicus,LSA)的关系目前尚存在争议,两者的临床表现和组织病理特征相似,均是以皮肤纤维化为特征的慢性炎症性皮肤病,猜测此两种疾病是同一疾病谱的两种不同表现形式。硬斑病的诊断主要根据病史、临床表现、组织病理学检查。硬斑病早期治疗效果较好,治疗主要包括局部治疗、系统用药、光疗、物理治疗等。桥本氏甲状腺炎(Hashimoto’S thyroiditis,HT)又称慢性淋巴细胞性甲状腺炎,是最常见自身免疫性甲状腺疾病。硬斑病和桥本均属自身免疫性疾病,且病因均可能与外界环境、自身免疫、遗传等有关,免疫性疾病之间可相互关联,硬斑病患者患自身免疫性疾病概率较正常人高,与硬斑病相关的自身免疫性疾病包括糖尿病、桥本氏甲状腺炎、Graves病、白癜风、溃疡性结肠炎、银屑病等。硬斑病等自身免疫性疾病的病因以及相互之间的关联机制有待明确。病例报告:例1女,64岁,颈前、躯干和双胫皮疹5年,会阴部皮疹伴瘙痒4年,既往桥本氏甲状腺炎病史9年。体检:甲状腺I度肿大,质韧,无突眼、胫前粘液水肿等表现。颈部、前胸淡红色斑片,后背部硬化萎缩,会阴部瓷白色斑块。组织病理提示:(前胸)硬斑病,(外阴)硬化性萎缩性苔藓。例2女,例1的妹妹,55岁,左乳下及腹部皮肤逐渐硬化萎缩4年,既往桥本氏甲状腺炎病史3年。体检:甲状腺I度肿大,质韧,无突眼、胫前粘液水肿等表现。左乳下、腹部和后背中央皮肤色素减退,硬化萎缩。组织病理提示:硬斑病。根据临床特点、组织病理、过碘酸-雪夫染色和甲状腺功能检查,2例均诊断为硬斑病合并桥本氏甲状腺炎。结论:首次报道了同一家族中姐妹同患硬斑病合并桥本氏甲状腺炎病例。从本报道家系谱中表明硬斑病和桥本氏甲状腺炎可能具有遗传性,自身免疫性疾病在同一家族、同一患者可同时并发,提示临床在诊断硬斑病时,应注意检测甲状腺功能并注意有无其他自身免疫性疾病病史和家族史。
[Abstract]:Background: (morphea), also known as localized scleroderma LS, is an autoimmune disease characterized by dermis thickening or sclerosis with subcutaneous fat deficiency. Women are more common, its exact cause is not clear, may be related to external factors, heredity, autoimmunity and so on. Although scleroplakia does not involve visceral organs, there is no Reynolds phenomenon or changes in nailfold capillaries, but LS can also involve subcutaneous fat, fascia, muscles and bones, affecting or even disabling joint activity. Neurological and ocular complications can occur in the head and face. It is divided into 5 subtypes: localized hard spot disease, linear hard spot disease, generalized hard spot disease, systemic sclerosing hard spot disease and mixed hard spot disease. The relationship between sclerosing sclerosus and lichen sclerosus (Lichen sclerosus et atrophicus) is still controversial. Their clinical manifestations and histopathological features are similar. Both of them are chronic inflammatory dermatosis characterized by skin fibrosis. It is assumed that the two diseases are two different manifestations of the same disease spectrum. The diagnosis of scleroplakia is mainly based on the history, clinical manifestation and histopathological examination. The early treatment of hard spot disease includes local treatment, systemic medication, phototherapy, physical therapy and so on. Hashimoto's thyroiditis (Hashimoto's thyroiditis HT), also known as chronic lymphocytic thyroiditis, is the most common autoimmune thyroid disease. Scleroplakia and Hashimoto are autoimmune diseases, and the etiology may be related to external environment, autoimmunity, heredity and so on. The incidence of autoimmune diseases in patients with scleroplakia is higher than that in normal subjects. Autoimmune diseases associated with scleroplakia include diabetes, Hashimoto's thyroiditis, Graves' disease, vitiligo, ulcerative colitis, and psoriasis. The etiology and mechanism of autoimmune diseases such as scleroplakia need to be clarified. Case report: case 1 female, aged 64 years, 5 years old with anterior cervical, trunk and double shank rash, perineal rash with pruritus for 4 years, and previous history of Hashimoto's thyroiditis for 9 years. Physical examination: thyroid I degree swelling, quality tough, no exophthalmos, anterior tibial mucus edema and other manifestations. Neck, anterior chest light red spot, posterior back hardening atrophy, perineum porcelain white plaque. Histopathological cues: hard spot disease (vulva) sclerosing atrophic lichen. Case 2 female, sister of case 1 55 years old, left submammary and abdominal skin sclerosis and atrophy for 4 years, previous history of Hashimoto's thyroiditis for 3 years. Physical examination: thyroid I degree swelling, quality tough, no exophthalmos, anterior tibial mucus edema and other manifestations. Hypopigmentation of the skin in the left lower breast, abdomen and the center of the back, sclerosis and atrophy. Histopathological cues: hard spot disease. According to clinical features, histopathology, periodate-Scheffer staining and thyroid function examination, 2 cases were diagnosed as hard spot disease with Hashimoto's thyroiditis. Conclusion: a case of Hashimoto's thyroiditis with hard spot disease among sisters in the same family is reported for the first time. From the pedigree of the Taoist family of this newspaper, it is suggested that hard spot disease and Hashimoto's thyroiditis may be hereditary, that autoimmune diseases are in the same family and the same patient can be concomitant at the same time, suggesting that clinical diagnosis of hard spot disease, Attention should be paid to the detection of thyroid function and to the history of other autoimmune diseases and families.
【学位授予单位】：大连医科大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R581.4;R593.25

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