角层下脓疱性皮病合并结缔组织病二例

报道2例角层下脓疱性皮病，皮损均表现为红斑基础上的水疱、脓疱，组织病理学特征为角层下脓疱，直接免疫荧光阴性；脓疱疱液细菌培养阴性。患者1，女，53岁，合并类风湿关节炎。口服阿维A，糖皮质激素和羟氯喹治疗。患者2，女，69岁，合并干燥综合症及自身免疫性肝炎，予糖皮质激素及米诺环素治疗。2例患者均好转。     

Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab

A 59-year-old woman presented with a painful, pruritic eruption that had commenced as an erythematous, dry patch on the upper back but progressed to erythroderma. Examination revealed orange-tinged erythroderma, scalp scaling, ectropion, palmoplantar keratoderma and nail changes. A diagnosis of type I adult-onset pityriasis rubra pilaris was made, and a subsequent skin biopsy was consistent with this. She was treated with a number of topical and systemic agents with minimal improvement or major side-effects. The patient was then treated with intravenous infliximab 5 mg/kg. She improved dramatically within 2 weeks and was no longer erythrodermic. Five further infusions resulted in additional improvement. Methotrexate was briefly added to the regime, but was ceased owing to nausea. Topical tar and keratolytics were used on the scalp. The patient was left with minimal disease activity and was maintained on emollients.     

白斑汤治疗178例白癜风疗效观察

目的观察蔡瑞康教授白斑汤治疗白癜风的临床疗效及临床经验。方法对入选每位患者选取面积最大或皮损面积最集中的部位进行观察,在门诊,按设计的表格进行定期观察,并作好详细记录(主要包括靶皮损面积、自觉症状、不良反应等),按拟定标准进行疗效评估,每2月为1疗程,共观察3个疗程。结果治疗6个月后,总有效率66.6%,治愈率9.0%,其中头颈部疗效最好,总有效率82.5%,治愈率11.6%,躯干部次之,总有效率61.9%,治愈率5.3%,四肢部疗效最低,总有效率25.0%,治愈率12.5%;按证型分肝肾不足型的总有效率为74.4%,高于气滞血瘀型的54.7%。结论白斑汤治疗白癜风适用于大多数白癜风患者。     

Adult Onset of Xanthelasmoid Mastocytosis: Report of a Rare Entity

Xanthelasmoid or pseudoxanthomatous mastocytosis is an extremely rare variant of diffuse cutaneous mastocytosis. Herein, we describe an adult male with cutaneous mastocytosis showing multiple widespread yellowish ovoid papules like eruptive xanthoma. A 60-year-old male visited our outpatient clinic with a 1-year history of generalized yellowish, ovoid, and skin color papular eruption located on the trunk, groin, extremities, with the modest pruritus. Vital signs were stable, and Darier''s sign was negative. No other subjective and objective signs were detected during the examination. No abnormality was detected in his diagnostic laboratory tests. Skin biopsy was taken, and histopathologic examination revealed proliferation of mast cells with ovoid and spindle nuclei with distinct cytoplasm borders around the capillaries, which was compatible with mastocytosis. Antihistamine was prescribed for pruritus control which was successful, but eruptions were persistent, and even 1-year phototherapy was not useful.     

先天性黏液型隆突性皮肤纤维肉瘤一例并文献复习

先天性DFSP临床罕见,其临床表现可类似血管性病变。黏液型先天性隆突性皮肤纤维肉瘤(congenital dermatofibrosarcoma protuberans,DFSP)是DFSP独特而罕见的亚型,具有其特征性病理组织学改变,生物学行为属交界恶性/潜在低度恶性。先天性黏液型DFSP更为罕见,国外仅有2例散发病例报道。本文报道一例并回顾相关文献。患儿,女,1岁9个月。出生后即有背部肿物,曾于外院误诊为血管瘤,予盐酸噻吗诺尔眼药水外敷治疗1年余,效果不佳。肿物不断增大,就诊我科后,予手术切除肿物并行病理组织学及免疫组织化学检查,诊断为先天性黏液型隆突性皮肤纤维肉瘤。考虑病理回报底切缘最近距离仅0.25 mm,根据指南行肿瘤边缘1 cm扩大切除治疗,随访2年未发现复发及转移。     

Hypergammaglobulinemic Purpura Associated with Sjögren's Syndrome and Chronic C Type Hepatitis

Although hypergammaglobulinemic purpura usually occurs secondarily on the lower legs in several disorders, purpura has also recently been reported to be associated with chronic C type hepatitis (HCV). To define the differences in the clinical, histological and laboratory findings and the prognosis of hypergammaglobulinemic purpura associated with those two disorders, we examined ten patients with hypergammaglobulinemic purpura, 6 patients with hypergammaglobulinemic purpura associated with Sjögren's syndrome (SjS) and 4 patients with hypergammaglobulinemic purpura with chronic C type hepatitis. Five of 6 patients with SjS were female. Attacks of purpura occurred in the pretibial area in all cases. Triggering factors included long walks and prolonged standing. The mean duration of attacks was 6.4 days. No systemic manifestations were associated. Anti-Ro/SS-A and anti-La/SS-B antibodies were expressed in one case. Spontaneous regression was noted in all cases; however, recurrence was noted in one. On the other hand, all the 4 patients with hypergammaglobulinemic purpura associated with HCV were men. Purpura was indurated in a few cases. Involved sites included the knee, forearm, abdomen and thigh in addition to the lower leg. The mean duration of attacks was 12.6 days. Recurrent purpura was noted in one case. Cryoglobulin was positive in three cases. In one patient with severe recurrent purpura, attacks stopped with prednisolone 10 mg/day. Histologically, leukocytoclastic vasculitis was detected in three cases associated with SjS and two cases with HCV. In conclusion, hypergammaglobulinemic purpura associated with HCV appears to occur unilaterally with a sex predilection for men and the manifestations last longer than those associated with SjS. Severe palpable purpura was also noted in association with HCV; systemic prednisolone resulted in good control.     

Spiradenocarcinoma with low-grade basal cell adenocarcinoma pattern: report of a case with varied morphology and wild type TP53

We present a patient with a 2-cm spiradenocarcinoma of the left arm resembling low-grade salivary gland basal cell adenocarcinoma. In addition to showing attributes of conventional spiradenoma, the benign component showed prominent areas of cystic change with focal apocrine differentiation, glands with and without mucinous differentiation, clear cell change and focal adenoid cystic carcinoma-like areas. The malignant component was composed of nodules of basaloid cells arranged in sheets with variable tendency to luminal differentiation. The nuclear atypia was low-grade, and the mitotic index was high in the malignant component (to 8/10 high power fields). Immunohistochemically, there was diffuse but variable positivity for cytokeratin 7 in both the benign and malignant components. Epithelial membrane antigen was focally positive, highlighting cells with ductal (luminal) differentiation. Expression of p63 was observed in 50 and 80% of the cells in the benign and malignant components, respectively. Calponin was negative. The proliferative index (MIB-1/Ki-67) was <3% in the benign component and up to 10% in the malignant component. Although the malignant component displayed patchy areas with nuclear p53 immunoreactivity with variable intensity, no mutation in the TP53 gene was identified.     

The role of selected neuropeptides in pathogenesis of atopic dermatitis

Background Atopic dermatitis (AD) is an inflammatory skin disease of a chronic course. The role of neuropeptides in pathogenesis of this disorder is probably not crucial; however, there is evidence that these substances influence the development and course of AD. Objective The aim of this study was to evaluate the plasma level of substance P, neuropeptide Y (NPY) and calcitonin gene related peptide (CGRP) in AD patients during exacerbation and remission of the disease. Material and methods Forty‐nine patients with AD, aged 17 to 56 years, participated in the study. Among this group, there were 25 males (51%) and 24 females (49%). The disease lasted from 1 to 55 years. The severity of the disease was assessed with SCORAD index. The severity of pruritus was evaluated with Visual Analog Scale and a specially designed questionnaire. Neuropeptides plasma level was detected with radioimmunoassay. Results Substance P plasma level in AD patients during exacerbation and remission was significantly higher than in the control group. There was a negative correlation between substance P plasma level and total IgE level. CGRP plasma level during exacerbation of AD was significantly lower than in healthy controls and increased in the remission. Significantly higher CGRP concentration was observed in patients suffering from severe pruritus; however, both in patients with more and less severe pruritus, CGRP plasma level was lower than in controls. Higher CGRP plasma level was also observed in patients with more severe disease. NPY plasma level in patients with AD was significantly increased both during exacerbation and remission. During remission of AD, NPY concentration was higher than during exacerbation.     

基因芯片检测人乳头瘤病毒23种亚型的应用评价

目的了解基因芯片检测性病门诊患者HPV23种亚型感染的临床应用价值。方法利用标记有生物素的HPV通用引物进行PCR扩增,扩增产物变性后与固定在尼龙膜上23种HPV特异性分型探针进行反向斑点杂交检测HPV亚型,同时采用荧光定量PCR检测HPV6/11和HPV16/18作比较。结果200例患者标本中,基因芯片阳性率47.04%,其中单一亚型感染63例,重叠感染31例。共检出亚型15种,6/11型的总检出率为38.5%,16/18型的总检出率为7.5%;荧光定量PCR阳性率40.0%,其中6/11总检出率为37.0%,16/18总检出率为7.0%。以基因芯片为基准,荧光定量PCR漏诊率为17.02%;以荧光定量PCR为基准,基因芯片漏诊率为2.50%。结论基因芯片一次试验可联合检测多种HPV亚型感染并分型,对研究性病门诊患者HPV感染型别的分布,提高由HPV各亚型引起的癌症病变的防治水平具有重要意义。     

复方氟米松软膏与丙酸氯倍他索乳膏治疗慢性湿疹和神经性皮炎疗效对比研究

[摘要] 目的 观察奥深治疗慢性湿疹和神经性皮炎的临床疗效和不良反应。方法 151例慢性湿疹和神经性皮炎患者随机分为奥深治疗组76例（其中慢性湿疹45例，神经性皮炎31例），丙酸氯倍他索乳膏对照组75例（其中慢性湿疹43例，神经性皮炎32例），分别观察治疗后1、2、3周的临床疗效和不良反应。结果 治疗组和对照组治疗1周后的临床痊愈率分别为13.16 %和6.67%，两组比较无统计学差异（P>0.05），治疗组治疗后2、3周的临床痊愈率分别为53.95%和63.16%，对照组治疗后2、3周的临床痊愈率分别为28.00%和37.33 %，两组比较差异有统计学意义（P<0.05）；治疗组治疗后1、2、3周的临床有效率分别为43.42%，84.21%和92.11%，对照组治疗后1、2、3周的临床有效率分别为18.67 %，60.00%和69.33%，两组临床有效率比较差异均有统计学意义（P<0.05）。且未见有明显临床意义的不良反应。结论 奥深治疗慢性湿疹和神经性皮炎的疗效优于丙酸氯倍他索乳膏，无明显的不良反应，是治疗慢性湿疹和神经性皮炎较为理想药物。     

蕈样肉芽肿的端粒酶活性与细胞增殖指数相关性的研究

目的：检测蕈样肉芽肿（MF）的端粒酶活性和细胞增殖情况，探讨它们在MF肿瘤发生机制中的作用及关系。方法：使用免疫组化法检测增殖细胞核抗原（PCNA）的表达情况；聚合酶链反应－酶联免疫吸附测定法检测端粒酶活性。结果：肿瘤期MF、斑片期MF、扁平苔藓和正常皮肤的PCNA细胞增殖指数分别为22．8、12．1、8．8、1．7和0；端粒酶活性的A值分别为0．737、0．313、0．240、0．045和0．044。端粒酶阳性的MF的PCNA细胞增殖指数高于端粒酶阴性的MF。结论：端粒酶活性存在于大部分MF标本中，端粒酶活性与MF的恶性程度及分期有关。端粒酶活性与细胞增殖指数有关，提示端粒酶的的活化可能为肿瘤细胞的无限增殖提供了先决条件。     

万乃洛韦联合紫外线照射治疗玫瑰糠疹疗效观察

目的观察万乃洛韦联合紫外线照射治疗玫瑰糠疹的疗效。方法将93例患者随机分为两组,治疗组60例口服万乃洛韦300mg 2次/d,连续10天;对照组33例口服左西替利嗪10mg 1次/d,维生素C 0.2g 3次/d,葡萄糖酸钙1.0g 3次/d,连续10天。两组均同时隔日一次局部紫外线照射。结果治疗组有效率为83.33%,对照组为57.57%,二者差异有显著性(P<0.01)。结论万乃洛韦联合紫外线照射治疗玫瑰糠疹的疗效好。     

隐性梅毒患者中神经梅毒患病率分析

目的 探讨广州市神经梅毒流行情况,比较未经治疗隐性梅毒患者和血清固定的隐性梅毒患者中神经梅毒的患病率.方法 以横断面研究的方法,对初次诊断为隐性梅毒患者(未治疗或经治疗后出现血清固定)抽取脑脊液,进行性病研究实验室试验,筛查神经梅毒患者.结果 未经治疗隐性梅毒患者中有21.98%(60/273)为神经梅毒,其中10.99%(30/273)为无症状神经梅毒,10.99%(30/273)为有症状神经梅毒.经抗梅毒治疗血清固定隐性梅毒患者83例中1.20%(1/83)符合神经梅毒诊断,为无症状神经梅毒病例.在未经治疗隐性梅毒组中,无神经梅毒病例年龄与有神经梅毒病例年龄差异有统计学意义,前者年龄低于后者.结论 在抗梅毒治疗后产生梅毒血清固定患者神经梅毒发生的可能性相对未经治疗的隐性梅毒小,神经梅毒仍主要发生于未经治疗的潜伏梅毒人群中,提示未经治疗的隐性梅毒是神经梅毒发生的主要危险因素.     

DLQI测量寻常型痤疮及白癜风患者生活质量的研究

目的比较寻常型痤疮患者与白癜风患者的生活质量。方法采用中文版皮肤病生活质量指标（delmatolo-gylifequalityindex,DLQI）对门诊寻常型痤疮患者与白癜风患者进行调查,并检测量表的信度和效度。结果寻常型痤疮患者DLQI得分为8．68,白癜风组得分为6．34．除条目1、条目3以及条目7,痤疮组得分高于白癜风组外,其余7个方面两组得分均无差别。DLQI量表测量痤疮与白癜风均具有较好的信度和效度,但条目1不能在白癜风患者间形成有效地区分度一结论寻常型痤疮患者的生活质量低于白癜风患者。DLQI量表用于测量白癜风患者的生活质量时应进行修订。     

新生儿皮肤再生不良1例

报告1例新生儿皮肤再生不良,患儿男,出生1h,生后即被发现左上肢皮肤缺损,分娩时无外伤史,给予0.1%利凡诺溶液及莫匹罗星软膏外用,10d后缺损处被新生组织填充,表面光滑,略凹陷,颜色较周围正常皮肤暗。     

合并HIV感染的银屑病患者12例临床分析

目的探讨合并HIV感染的银屑病患者临床特征。方法收集2015年1月-2019年12月本院收治的12例合并HIV感染的银屑病患者的临床资料,进行回顾性分析。结果12例患者中,寻常性银屑病6例,关节病型银屑病1例,脓疱型银屑病1例,红皮病型银屑病4例;男女比例5∶1;银屑病病史平均(7.88±7.86)年,入院时病情加重平均(1.00±0.57)个月。银屑病皮疹累及头面部6例(50.00%)、躯干12例(100%)、四肢9例(75.00%)、掌跖及指(趾)甲6例(50.00%);伴关节疼痛4例(33.33%)。患者头面、躯干、四肢处皮疹表现为典型的银屑病红斑鳞屑疹,与HIV阴性患者未见明显差异。银屑病PASI评分为(16.67±10.70)分。本次研究中接受治疗的9例患者,CD4⁺T淋巴细胞均低于正常值,CD4/CD8均低于正常范围,CD4⁺T淋巴细胞计数越低,病情越重。9例患者均予以复方甘草酸苷静滴,配合外用糖皮质激素药膏等治疗,其中6例患者口服阿维A胶囊20~30 mg/d,治疗有效,但皮疹未完全消退,无死亡病例。结论本组合并HIV感染的银屑病患者12例中,男性居多,以寻常性银屑病为主;患者掌跖及指(趾)甲更易受累;CD4⁺T淋巴细胞计数越少,银屑病皮疹越重;此类患者早期治疗方案与一般银屑病患者相仿,皮疹难以完全消退,但存活率高。     

系统性红斑狼疮并发脓疱性银屑病和乙型肝炎

报告1例系统性红斑狼疮(SIJE)并发脓疱性银屑病和乙型肝炎.患者女,25岁.2个月前确诊为SLE,在给予糖皮质激素治疗过程中突然出现全身皮肤红斑、鳞屑、脓疱,伴瘙痒、发热,诊断为脓疱性银屑病.入院后又发现患有乙型肝炎.     

A case of Adamantiades‐Behçet disease with ischemic optic neuritis (posterior optic neuropathy)

Adamantiades‐Behçet disease (ABD) may present with cutaneous and ophthalmologic finings. A 29‐year old woman complained of fever and general fatigue, along with erythema nodosum and vesiculo‐pustular lesions on the legs, acneiform lesions, genital ulcerations and painful oral ulcers. She also complained of reduced visual acuity, visual disturbance and blurred vision in the left eye. Her left visual acuity was 6/20. Light reflex in the left eye was reduced. The relative afferent pupillary defect (RAPD) was positive in the left eye where a central scotoma was present. The vitreous was clear; the optic disc, macula, retina and iris were all normal. Uveitis was not observed. The patient was diagnosed with ischemic optic neuritis (posterior optic neuropathy) with ABD. Histopathological findings taken from a blister on the leg showed subepidermal bulla, dense dermal neutrophil infiltration, and extravasation of erythrocytes, suggesting leukocytoclastic vas‐culitis. She was treated orally with high‐dose corticosteroids (methylprednisolone 500 mg/d) for three days. Her general condition and ophthalmic symptoms resolved completely. Optic neuropathy with ABD is very rare; we know of two previous cases [ 1 , 2 ] of ABD with ischemic posterior optic neuritis.     

界线类偏瘤型麻风伴I型麻风反应一例

患者，男，27岁。全身水肿性红斑、斑丘疹伴痛，高热1周。组织液抗酸染色查菌BI 3.40。组织病理检查示：表皮变薄，表真皮间无浸润带，真皮内成团淋巴细胞、组织细胞、泡沫细胞等浸润，累及脂肪层和附属器。2年前患者曾诊断为BL麻风，抗麻风联合化疗治疗1年。     

维布妥昔单抗治疗Sézary综合征一例

患者,男,41岁.全身弥漫性红斑伴严重瘙痒1年,多发淋巴结肿大3个月.PET/CT符合淋巴瘤;淋巴结病理:非霍奇金T细胞淋巴瘤,伴不规则、扭曲、脑回样核;皮肤病理:非霍奇金T细胞淋巴瘤,呈蕈样肉芽肿改变.结合患者症状、体征、影像学、病理及免疫组化结果,以及外周血T细胞基因重排均为阳性,诊断为Sézary综合征.给予化疗...