自身免疫性多腺体综合征Ⅳ型：胸腺瘤、白癜风、重症肌无力、系统性红斑狼疮、寻常型天疱疮及普秃并发一例  

Autoimmune polyglandular syndrome type IV： thymoma, vitiligo, myasthenia gravis, systemic lupus erythematosus, pemphigus vulgaris and alopecia universalis successively occurred in a patient

作　　者：钱明彤 刘佳玮[1] 刘薇[1] 马东来[1] Qian Yuetong,Liu Jiawei,Liu Wei,Ma Donglai(Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院皮肤科,北京100730

出　　处：《中华皮肤科杂志》2018年第10期733-736,共4页Chinese Journal of Dermatology

基　　金：北京市自然科学基金（7172167）

摘　　要：患者女，62岁，因全身多发白斑16年，头皮、躯干、上肢红斑、水疱8年余、加重1年于2017年11月30日就诊。既往史：1996年行胸腺瘤手术切除术，2004年患重症肌无力。皮肤科情况：头皮、面部、胸部、上肢散在分布大小不一的红斑，部分表面结痂，尼氏征阳性；全身散在分布大小不一的色素减退斑和色素脱失斑；双手大面积色素脱失，指甲甲板萎缩；头发、眉毛、睫毛、腋毛、阴毛均缺失。实验室检查：抗核抗体（IgG型）均质型阳性，滴度1：640，抗双链DNA抗体276IU／ml，狼疮抗凝物1．62，抗Dsg-1抗体〉150U／ml，抗Dsg-3抗体91U／ml。取背部水疱行组织病理学检查，HE染色示表皮内裂隙性水疱，可见棘层松解细胞，基底膜上可见单层基底细胞，真皮浅层血管周围少量淋巴细胞浸润。该患者相继出现胸腺瘤、白癜风、重症肌无力、系统性红斑狼疮、寻常型天疱疮及普秃，诊断为自身免疫性多腺体综合征Ⅳ型。由于各个内分泌腺的自身免疫性疾病常逐渐发生，应监测本例患者相关激素及抗体的变化，以早期诊治新发的自身免疫性疾病。On November 30th, 2017, a 62-year-old female patient was admitted to the hospital because of multiple depigmented patches all over the body for 16 years, as well as erythema and blisters on the scalp, trunk and upper extremities for more than 8 years, which had been aggravated for 1 year. The patient underwent surgical resection of thymoma in 1996, and developed myasthenia gravis in 2004. Skin examination showed scattered erythemas of various sizes on the scalp, face, chest and upper extremities, with crusts on the surface of some erythemas and positive Nikolsky＇s sign. Hypopigmented macules mingled with depigmentated macules of various sizes were scattered all over the body. A large area of depigmentation was observed on both hands, and the nail plates were atrophic. Hairs, eyebrows, eyelashes, axillary and pubic hairs were abseent. Laboratory examination showed the presence of homogeneous IgG antinuclear antibody with a titer of 1： 640, anti- double stranded DNA antibody （276 IU/ml）, lupus anticoagulant （1.62）, anti-Dsgl antibody （ 〉 150 U/ml） and anti- Dsg3 antibody （91 U/ml）. Histopathological examination of dorsal blister tissues and hematoxylin-eosin staining showed fissured blisters and acantholytic cells in the epidermis, monolayer basal cells on the basilar membrane, and perivascular infiltration of a few lymphocytes in the superficial dermis. The patient successively developed thymoma, vitiligo, myasthenia gravis, systemic lupus erythematosus, pemphigus vulgaris and alopecia universalis, so a diagnosis of autoimmune polyglandular syndrome type 1V was made. Since autoimmune- mediated endocrinopathies are prone to occur successively, changes of related hormones and antibodies in the patient should be monitored for early diagnosis and treatment of newly emerging autoimmune diseases.

关 键 词：胸腺瘤 白癜风 天疱疮 重症肌无力 红斑狼疮 系统性 秃发 自身免疫性多腺体综合征 

分 类 号：R58[医药卫生—内分泌]