大疱性系统性红斑狼疮一例

患者女,43岁,全身皮肤黏膜水疱、红斑3年余,加重伴肩关节痛2个月.3年前,患者双手背出现米粒至花生大小的水疱,伴瘙痒,逐渐累及头皮、面颈、躯干、四肢和口腔黏膜,口腔内水疱破溃后形成溃疡,可自愈,皮肤水疱痊愈后遗留色素减退或色素沉着斑,皮疹反复发生.近2个月皮损加重,伴双侧肩关节疼痛.病程中轻度多饮、多食、多尿,近1年体重减轻9 kg.既往史:8年前面部出现红斑,外院诊断红斑狼疮,应用糖皮质激素治疗1年,皮疹消退后停药,未定期复查.体检:各系统检查无异常.皮肤科检查:下颌、颈前可见不规则淡红斑,其上密集水疱,躯干及四肢散在水疱,水疱粟粒至花生大小,疱壁紧张,疱液淡黄色,Nikolsky征阴性(图1);全身皮肤散在大小不一色素减退及色素沉着斑;口腔内颊黏膜见2处指甲大小淡红斑.双侧肩关节运动时疼痛,无压痛及叩痛,双上肢外展上举不能至肩水平面.实验室检查:血常规正常.     

大疱性类天疱疮伴食管恶性黑色素瘤1例

报道大疱性类天疱疮（BP）伴食管恶性黑色素瘤（PMME）1例。患者女,61岁,既往PMME史3年余,现黏膜破溃4个月,加重伴周身红斑水疱0.5个月。皮肤科检查：面部出现水肿性红斑,红斑基础上覆厚腻黑痂;躯干、四肢泛发水肿性红斑、丘疹,红斑及正常皮肤基础上可见粟粒至甲盖大小水疱,疱壁紧张,疱液清亮,部分水疱破溃渗出结痂,尼氏征阴性;口腔可见溃疡;外阴部黏膜糜烂。组织病理及免疫荧光检查均符合BP。诊断：BP伴发PMME。     

偏瘤型界线类麻风合并类天疱疮一例

患者女,53岁。躯干及四肢起红斑、水疱,伴剧痒5个月到我院皮肤科门诊。5个月前无明显诱因双下肢出现红斑、水疱,并逐渐波及躯干和上肢,伴有剧烈瘙痒。水疱破溃后可自行愈合,留暗红色色素沉着斑。曾在当地医院服中药治疗无效。皮肤科情况：躯干及四肢散在分布红色水肿性斑片,部分红斑上有黄豆大水疱,疱壁紧张,Nikolsky征阴性,口腔黏膜无损害。临床拟诊为类天疱疮,给予泼尼     

大疱性系统性红斑狼疮合并狼疮性肾炎1例

正1临床资料患者,女,19岁。因面颈部、躯干水疱、大疱、结痂伴痒2周就诊。2周前,患者无明显诱因胸部出现绿豆至蚕豆大小水疱、大疱,疱液澄清,疱壁紧张,不易破溃,未治疗。后皮损加重,逐渐累及至颈部及颜面部,口腔黏膜出现溃疡,无明显疼痛及瘙痒。患者自行搔抓后水疱破溃,流出淡黄色液体,随后皮损干燥结痂,自觉轻微痒痛。自发病以来,患者精神、     

寻常型天疱疮并甲损害一例北大核心CSCD

患者女,32岁,因反复口腔、外阴溃疡1年半,复发伴全身红斑、水疱,甲损害1周于2014年5月24日入院.患者于2012年12月初无明显诱因下舌和唇黏膜开始出现溃疡,在当地口腔医院诊断为口腔炎,具体用药不详,口腔溃疡难以愈合.2012年12月下旬患者外阴出现溃疡,诊断为Behcet综合征,予醋酸泼尼松片（最大剂量为50 mg/d）口服,口腔及外阴溃疡可愈合.在医生指导下逐渐将泼尼松减量至5 mg/d维持治疗.1周前患者口腔、外阴再次出现糜烂,躯干、四肢陆续出现红斑,红斑基础上起绿豆大小的水疱,散在分布,水疱轻轻摩擦则破溃,露出潮红的糜烂面,痒痛明显.     

PD-1单抗致相关大疱性类天疱疮1例

患者男,70岁,口腔溃疡6个月余,四肢躯干红斑丘疹3个月,水疱1个月余。患者既往因肾透明细胞癌行帕博利珠单抗治疗2年。皮肤科情况：躯干四肢见外观正常皮肤或红斑基础上散在紧张性黄豆至蚕豆大小水疱,疱液澄清,尼氏征阴性,部分破溃结痂,前胸及腹部见数个蚕豆大小血疱,下唇见一直径约为0.6 cm大小水疱,左侧颊黏膜见一浅溃疡。皮损组织病理示：表皮下水疱,疱顶为全层表皮,疱底为真皮乳头,疱内有浆液性渗出,疱内及真皮浅层大量嗜酸性粒细胞浸润。直接免疫荧光：基底膜带IgG、C3、C4线状沉积。诊断：大疱性类天疱疮(PD-1单抗相关)。     

药物诱发的大疱性类天疱疮一例

患者,女,20岁。全身皮肤红斑水疱伴瘙痒1个月。1个月前患者因口服“氨酚黄那敏颗粒”后全身出现红斑水疱。皮肤专科查体：上唇、颈部、胸背、腋下、外阴及双大腿内侧见红斑,其上多发黄豆至蚕豆大小的水疱、大疱,疱壁紧张,部分水疱破溃,上附淡黄色痂,尼氏征(-),部分区域可见虹膜样改变,口腔黏膜、舌部受累,可见散在糜烂、水疱。实验室检查、组织病理、直接免疫荧光结合盐裂试验,诊断为药物诱发的大疱性类天疱疮。经甲泼尼龙等治疗1个月后痊愈停药。     

寻常型天疱疮并发多发性脑脓肿1例

临床资料患者男,45岁.因全身反复发生水疱、糜烂、结痂1年,加重2个月于2010年6月26日入院.1年前无明显诱因出现口干,饭量、尿量增多,当时未介意及治疗,50多天后觉咽部进食时疼痛不适,应用环丙沙星、头孢曲松钠等抗生素治疗10天症状无改善,且舌体两侧及颊黏膜出现糜烂、溃疡,应用中药(具体不详)治疗半月,口腔糜烂面逐渐扩大增多,影响进食,头面部、躯干、四肢相继出现数十个散在的绿豆至蚕豆大水疱,疱壁薄而易破溃,难愈合.     

接种新型冠状病毒疫苗后罹患Stevens-Johnson综合征一例

患者，女，31岁。双侧眼睑红肿9天，发热8天，全身皮肤红斑6天。患者发病前1天有第三剂新型冠状病毒疫苗接种史。皮肤科查体：面部红肿，双眼结膜明显充血，眼周、口周糜烂，上覆厚层血痂，口腔黏膜散在分布糜烂面，躯干、双上肢散在分布水肿性红斑，部分红斑中央可见水疱，疱壁紧张，尼氏征(-),左上臂可见约7 cm×5 cm大鲜红色糜烂面，外阴黏膜红肿、糜烂，少量渗出。结合临床表现诊断为Stevens-Johnson综合征。给予甲泼尼龙、环孢素等治疗，皮疹逐渐减轻。     

首发于咽峡部的大疱性类天疱疮1例

患者男,67岁。全身红斑水疱伴痒8天入院。5月前因咽部不适、痰中带血及淡黄色膜,以溃疡膜性咽峡炎抗炎治疗无效,纤维支气管镜检见气管、支气管粘膜充血,覆有淡黄色假膜,组织病理示气管粘膜慢性炎症,疑为天疱疮,予强的松30ｍｇ/ｄ治疗1月症状好转。8天前无明显诱因,上肢、腕部痒,并起红斑、水疱,延及躯干、四肢。入院查体:咽充血、覆有淡黄色假膜。全身皮肤散在红斑、水疱、大疱,疱壁紧张较厚,疱液清,尼氏征阴性。血、尿、粪常规及免疫指标正常。组织病理符合大疱性类天疱疮。治疗　强的松60ｍｇ/ｄ及对症治疗3天后无新起水疱,1月后皮损消退,…     

儿童获得性大疱性表皮松解症一例

报道1例儿童获得性大疱性表皮松解症.患者女,12岁,因全身反复水疱、大疱和糜烂面3个月入院.患者弱智,父母非近亲结婚,无家族史.入院时表现全身大片糜烂面,包括手足、膝关节周围和股臀部,同时躯干、四肢正常或水肿性红斑基础上紧张性水疱、大疱.治疗过程中突然发生躯干、四肢大量紧张性水疱.组织病理提示,表皮下水疱,免疫荧光结果见表皮基底膜带IgG和C3线状沉积,盐裂后沉积限于真皮侧.ELISA检测BP180和BP230阴性.采用大剂量糖皮质激素联合四环素口服取得满意疗效.

Abstract：

A case of epidermolysis bullosa acquisita (EBA) in childhood is reported. A 12-year-old girl was hospitalized for a 3-month history of recurrent blisters, bullae and erosions on the trunk and limbs. The girl had mental retardation but no family history of similar disorders. The marriage between her parents was not consanguineous. Physical examination on admission revealed large erosions with moderate oozing on the hands and feet, around the knees and on the buttock and thighs. There were scattered tense blisters and bullae arising in normal skin or edematous erythema on the trunk and limbs. During the treatment course the patient suddenly developed a number of tense blisters over the whole integument on the trunk and limbs. Skin biopsy showed subepidermal bullae with moderate perivascular infiltration of neutrophils and eosinophils. Direct immunofluorescence (DIF) revealed linear IgG and C3 deposition along the basal membrane zone, which was on the dermal side of salt-split skin on indirect immunofluorescence (IIF). ELISA detected no serum antiBP180 or -BP230 antibodies in the patient. A diagnosis of EBA was made. The patient was successfully controlled by intravenous steroids combined with oral tetracycline.     

新生儿线状IgA大疱性皮病

患儿男, 生后10 d, 因皮肤红斑、水疱6 d就诊入院。皮肤科检查:全身皮肤散在或融合分布红斑, 在正常皮肤或红斑基础上可见大小不等的紧张性水疱, 部分水疱破溃、糜烂;口腔黏膜可见血疱、红色糜烂面。组织病理检查示表皮下水疱, 疱内可见中性粒细胞及少量嗜酸性粒细胞。直接免疫荧光检查显示:沿基底膜带有均质型线状IgA和颗粒状C3沉积, IgG阴性。诊断:新生儿线状IgA大疱性皮病。给予营养支持、防感染等综合治疗后, 皮肤红斑、水疱消退, 黏膜损害减轻出院。患儿出院后16个月电话随访, 一般情况良好, 皮肤黏膜皮损消退、愈合, 无新发皮疹, 生长发育正常。     

重症多形红斑型药疹合并急性胰腺炎继发巨细胞病毒感染一例

报告1例牛黄解毒片所致重症多形红斑药疹合并急性胰腺炎及上消化道出血并继发巨细胞病毒感染。患者女,20岁。因口、眼、外阴黏膜糜烂、疼痛,伴发热4天,皮肤科检查：全身散在分布黄豆大小水肿性红斑,部分呈靶形,中央水疱或紫癜,眼结膜、口唇、口腔、外阴广泛性充血、糜烂、渗出,被覆白色膜样物质和黄色脓性分泌物。入院诊断重症多形红斑,入院后出现急性胰腺炎、上消化道出血,经及时救治后好转但出现不明原因持续发热,根据血常规、巨细胞病毒血清学结果诊断为巨细胞病毒感染,给予更昔洛韦治疗2周后痊愈。     

Multiple endocrine neoplasia type 2b associated with lichen nitidus

Multiple endocrine neoplasia (MEN) type 2B syndrome is an autosomal dominantly inherited endocrine disorder with rare skin manifestations. We report the case of a 19-year-old Turkish girl who presented with skin-colored flat papules scattered all over the trunk and extremities. Additionally, she had marfanoid habitus, thick lips, and multiple flesh-colored papules over the inner eyelids and oral mucosa. Histopathological examination of one of the trunk lesions was consistent with lichen nitidus. Her past medical history was significant for medullary thyroid carcinoma. Genetic testing showed a point mutation in exon 16 at codon 918 (M918T) in the RET proto-oncogene. Based on all these findings, MEN type 2B was diagnosed. To the best of our knowledge we report the first case of MEN type 2B associated with lichen nitidus.     

色素失禁症一例并文献复习

患儿,女,2个月。因躯干及四肢皮疹2个月就诊。皮肤科检查：躯干及四肢皮肤弥漫分布网状色素沉着斑。双股内侧、手腕部见暗红色大小不一丘疹、水疱,水疱壁厚,尼氏征阴性,部分水疱结痂。皮疹呈泼溅状分布。组织病理示：表皮角化过度,棘层增厚,表皮内见多房性水疱,疱液中可见大量嗜酸性粒细胞。真皮浅层毛细血管周围可见少量淋巴细胞及嗜酸性粒细胞浸润。诊断：新生儿色素失禁症。     

Das eosinophile Ulkus der Mundschleimhaut Differenzialdiagnose zu anderen oralen Ulzerationen

Eosinophilic ulcers of the oral mucosa represent a rare, self-limiting disease of unknown origin. Single lesions occur typically in the oral cavity or on the lower lip. Characteristically, they have a short history and resolve spontaneously. Diagnosis is verified by the histologic feature of a deep polymorphic inflammatory infiltrate with numerous eosinophils. The clinical aspect shows a usually nontender ulcer with a white or yellowish base and elevated indurated borders or a tumor with central ulceration. Lymphadenopathy is not found. Differential diagnoses include malignant tumors and the primary stage of syphilis. We present three cases recently diagnosed in our department to show the characteristics and the different appearances of the disease. A 74-year-old woman presented with an ulcer at the base of the tongue, a 59-year-old man had one on the lower lip, and in a 48-year-old man the lesion was atypically located at the edge of the mouth. Here we demonstrate the importance of knowledge of the disease, its course, and the characteristic histology to avoid troublesome diagnostic and therapeutic procedures.     

分别由平菇、黑木耳、金针菇诱发鞭挞样香菇皮炎各一例

【摘要】 报道分别由平菇、黑木耳、金针菇诱发鞭挞样香菇皮炎各1例。例1男,45岁,发病前8 h食用大量烧烤平菇,躯干及腋下和腹部出现多条鞭挞样红斑、丘疹,无自觉不适。例2女,33岁,食用凉拌新鲜黑木耳1.5 d后,发现后背多条鞭挞样红斑,轻度肿胀,表面密集分布粟粒大丘疹,自觉轻度瘙痒。例3女,54岁,进食金针菇72 h后,颈部、躯干及四肢近端可见条索状、鞭挞样水肿性红斑、丘疹。例3背部皮损组织病理: 表皮海绵水肿,表皮内水疱,真皮乳头水肿,真皮浅层血管扩张,红细胞溢出,扩张的血管周围以淋巴细胞为主的炎性细胞浸润,散在少数嗜酸性粒细胞。诊断：鞭挞样香菇皮炎。例1未处理,例2、例3外用丙酸氟替卡松软膏对症治疗,1周后皮损痊愈。     

Case of pemphigus vulgaris in oral mucosa showing an extension to the body surface with recurrent varicella

A 60-year-old female with pemphigus vulgaris limited to the oral mucosa being treated with prednisolone showed a widespread extension of lesions to the body and extremities over a short period. The eruptions on the body began as small vesicles that looked unlike the blisters of pemphigus vulgaris, and increased to large blisters and erosions compatible with pemphigus. A smear test of the vesicles in the early stage demonstrated multinucleated acantholytic keratinocytes, which were positively stained with an antivaricella zoster virus (VZV) antibody by the immunoperoxidase method. The widespread eruptions improved immediately after 5 days' administration of valaciclovir hydrochloride tablets and increased dosage of prednisolone, leaving tiny shallow scars and pigmentation. The anti-VZV antibody in the serum was not elevated, and there were no zosteriform eruptions noted during the course. We thought that widespread extension of the pemphigus vulgaris in this case was provoked by recurrent varicella, in a similar fashion to Koebner phenomenon.     

Nicorandil-induced leg ulceration without mucosal involvement

We report a case of leg ulceration occurring in a patient without mucosal ulcers, in whom nicorandil appeared to be the main aetiological factor. Having failed to heal on compression therapy, the ulcer rapidly improved and healed after the discontinuation of nicorandil. Most cases of nicorandil-induced ulcers reported in the literature develop on mucosal surfaces, including oral, vulval, perianal and peristomal ulcers. There are rare reports of cutaneous ulceration attributable to nicorandil, occurring concurrently with mucosal ulcers. To our knowledge, this is the first case of nicorandil-induced leg ulceration affecting the skin without mucosal involvement.     

Drug-induced Quincke's edema of the mouth mucosa - an analysis of 33 cases

Since literature only provides us with scarce information about occurrence and etiologic conditions of oro-pharyngeal Quincke's edema, we performed a retrospective evaluation of the medical records of 4,766 in-patients between the years 1970 and 1980 with registered drug-induced cutaneous and/or mucosal side effects. Among these cases there were 187 patients (= 3.92%) showing oral side effects, 33 of them with the diagnosis of Quincke's edema on the labial, oral or pharyngeal mucosa. 30 cases revealed clinical and/or historic data about concomitant occurrence of edematous lesions also in other body sites. With all patients drug intolerance was suspected by history, yet drug testings could only prove this assumption in 24 cases. Salicylates, analgetic compounds, barbiturate, pyrazolone, and penicillin are the main etiologic factors for oral Quincke's edema.