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.     

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。     

1%环吡酮胺软膏治疗浅部真菌病的疗效观察

目的 :评价环吡酮胺软膏治疗皮肤浅部真菌病的疗效和安全性。方法 :1 3 0例浅部真菌病分成二组 ,治疗组外用环吡酮胺软膏 ,对照组外用特比萘芬乳霜 ,体股癣疗程 2周 ,手足癣疗程 4周。结果 :治疗组 66例中治愈 42例 ,显效 1 8例 ,临床总有效率 90 91 % ,真菌清除率 89 3 9% ;对照组总有效率为 92 1 9% ,真菌清除率为 90 63 % ,两组疗效差异无显著性 (P >0 0 5 )。结论 :环吡酮胺软膏治疗皮肤浅部真菌病疗效显著且安全     

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。     

儿童朗格罕细胞组织细胞增生症50例临床分析

目的探讨朗格罕细胞组织细胞增生症(Langerhans cell histocytosis,LCH)的临床表现、分型、分级、实验室检查和治疗情况及预后随访。方法对本院(2002～2010)年收治的50例住院LCH患儿临床资料进行回顾性分析及随访。结果临床分型LS 20例,LSC 2例,EG 18例,中间型10例。分级I级16例,II级15例,III 9例,IV 10例。本组50例中,31例均进行了手术或联合化疗治疗,其中10例EG病例单纯手术,8例EG行手术切除+化疗。结论 LCH是一种反应性非肿瘤性增殖性疾病,病因不明,儿童多见,临床表现多样性,易误诊、漏诊,X线是其重要的辅助检查,皮损、软组织肿块、溶骨性病灶、肿大的淋巴结、肝脾等进行活组织病理检查是确诊本病的依据,如有可疑皮疹,需及时完善皮疹印片及皮肤活检以协助诊断。本病是一组异质性疾病,个体化治疗极为重要,患者的年龄、病变的范围及损害的部位是选择治疗的依据,目前尚无特异有效的治疗方法。     

Azathioprine hypersensitivity presenting as a neutrophilic dermatosis in a man with ulcerative colitis

We report a case of a 46-year-old man with ulcerative colitis being treated with oral prednisolone and azathioprine. Two weeks after the initiation of azathioprine he presented with fever, fatigue, myalgias and arthralgias and a painful cutaneous eruption that was most marked in a sun-exposed distribution. This was accompanied by loose, non-bloody diarrhoea. Histopathological assessment of a skin biopsy supported a diagnosis of a neutrophilic dermatosis. The azathioprine was temporarily withheld and oral prednisolone was increased as it was thought that the neutrophilic dermatosis was associated with the underlying ulcerative colitis. The patient's symptoms and cutaneous eruption resolved quickly and azathioprine was re-introduced. Within 24 h, systemic symptoms returned along with a florid recrudescence of his cutaneous eruption. This rapidly improved upon withdrawal of azathioprine.     

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.     

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.     

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.     

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。     

SLE患者外周血B细胞B7RP-1的表达及其与疾病活动度的相关性

目的 探讨SLE患者B细胞分化和B7相关蛋白-1(B7RP-1)在B细胞上的表达.方法 用三色流式细胞仪检测23例初发SLE患者和16例正常人对照浆细胞、记忆性B细胞、原始B细胞的百分比,并检测三类B细胞上B7RP-1的表达量;同时收集患者临床资料,进行SLE疾病活动度指数(SLEDAI)评分,分析疾病活动度与B7RP-1表达水平的关联.结果 活动期和非活动期SLE患者浆细胞所占比例较正常人对照显著增加(P＜0.01),且活动期高丁非活动期(P＜0.01);相对于正常人对照,活动期SLE患者记忆性B细胞所占比例减少(P＜0.01),非活动期同正常人对照组差异无统计学意义;原始B细胞所占比例三组间差异无统计学意义.SLE肾炎组浆细胞所占比例高于非肾炎组(P＜0.05),记忆性B细胞和原始B细胞两组差异无统计学意义.SLE患者B细胞B7RP-1表达(46.5l%)低于正常人对照组(63.75%),且三种类型B细胞B7RP-1表达量均低于正常人(P＜0.01),但活动期和非活动期患者B7RP-1的表达差异无统计学意义.SLE患者B7RP-1的表达同疾病活动度无父联(r=0.035,P＞0.05),狼疮肾炎和非狼疮肾炎组B7RP-1表达差异无统计学意义.结论 SLE患者外周血浆细胞所占比例增加,B7RP-1的表达下降,可能与SLE的发病机制有关.     

恶性萎缩性丘疹病一例

患者，女，58岁。躯干四肢散在红斑丘疹4个月余。皮肤科检查：躯干四肢散在红斑丘疹，丘疹中央可见瓷白色萎缩性斑片，边缘隆起伴红晕，伴腹痛。皮损组织病理示：表皮萎缩变薄，真皮浅中层胶原变性，血管周围散在小片状的淋巴细胞及组织细胞浸润。诊断：恶性萎缩性丘疹病。给予阿司匹林、双嘧达莫、雷贝拉唑钠、美沙拉嗪、阿嗪米特口服治疗1个月后腹痛症状缓解，全身皮疹颜色变淡。2个月后患者腹痛加重诊断为肠穿孔，术后无明显改善。4个月后去世。     

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。     

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。     

误诊为湿疹的挪威疥一例

患者，男，24岁。全身皮肤起丘疹伴瘙痒1年余就诊。当地医院曾按“泛发性湿疹”予抗过敏治疗，皮损未见好转。取皮疹鳞屑镜检见疥螨。诊断：挪威疥。经治疗痊愈。     

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。     

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.