计算机X线断层扫描图像分析颌面部深在性红斑狼疮一例

患者女,34岁,因面部皮损2年,于2012年1月5日来我院就诊.患者于2010年3月无明显诱因双侧面颊部、下颌处出现红斑、皮下硬结,无压痛,无明显自觉症状.近2年斑块面积及皮下硬结渐增大,同时伴有明显皮肤凹陷和萎缩.患者偶有肌肉疼痛,无光敏感、口腔溃疡、关节痛等其他症状.既往体健,否认面部外伤史和局部注射史,无系统性红斑狼疮、淋巴瘤等相关病史.家族中未见有直系亲属罹患本病.育有一子,现已10岁,身体健康.体检:各系统检查无异常.皮肤科检查:双面颊及颌下部皮肤深度凹陷,凹陷处皮肤萎缩、瘢痕,边缘仍有浸润性斑块,隆起呈弓状,暗红色,境界清楚,质硬,与皮肤粘连,不能移动(图1,2).     

萎缩性皮肤病

20 0 4 2 370　皮肤痘疮样斑状萎缩 1例 /陈永锋 (广东省皮肤性病防治中心 )… / /岭南皮肤性病科杂志 .- 2 0 0 3,10 (2 ) .- 12 3男 ,2 2岁 ,以躯干、四肢小片状皮肤萎缩 12年就诊 ,其姐有类似的皮肤萎缩。体检 :系统检查无异常。皮肤科情况 :整个胸背部和四肢遍布圆形或卵圆形的皮肤萎缩 ,肤色或淡白色 ,直径约 0 .3～ 0 .5cm,触之有松弛感。血尿常规均正常。皮肤组织病理显示表皮萎缩及真皮浅层弹力纤维消失。参 2　 (邓翠霞 )2 0 0 4 2 371　面部单侧萎缩合并颅脑病变 1例 /王娣 (北京大学第一医院皮肤科 ) / /临床皮肤科杂志 .- 2 0 0…     

结节萎缩性皮肤淀粉样变性

报告1例结节萎缩性皮肤淀粉样变性.患者女,60 岁.双股伸侧红褐色萎缩性斑块伴疼痛5 年.皮肤科检查:双股伸侧散在分布暗红至红褐色斑块及结节,有轻度萎缩、松弛,萎缩中心局部指压时可见疝样凹陷.皮损组织病理检查:表皮萎缩变薄,真皮全层、皮下组织及血管周围有大小不一均一红染、团块状的淀粉样蛋白沉积.真皮及皮下脂肪血管周围有较多的淋巴细胞、浆细胞浸润,其间散在少许嗜酸性粒细胞.结晶紫染色阳性,PAS染色可疑阳性.诊断:结节萎缩性皮肤淀粉样变性.     

进行性特发性皮肤萎缩1例

进行性特发性皮肤萎缩也称Pasini-Pierini皮肤萎缩(atrophoderma of Pasini-Pierini,APP)是一种特殊类型的皮肤萎缩性疾病.多发生青年女性,临床表现为境界清楚的萎缩性斑片,皮下静脉显露,病程中仅有萎缩而无水肿、硬化.现将我科诊治的1例报告如下:     

多发性肌炎、皮肌炎的诊断指南——日本厚生省特定疾患研究班制订

(一)诊断依据:1.主要症状:(1)肌肉症状:①急性、亚急性或慢性进行.②有时伴有肌痛.③四肢肌(特别是近位端肌肉),面肌,颈肌、咽肌、喉头肌等肌张力低下及肌萎缩,但要除外其他胶原病伴有的肌肉症状.(2)皮肤症状:面部、上胸部、四肢伸侧特别是关节背面等处,呈对称性.①紫红色的浮肿性红斑(特别是上眼睑).②毛细血管扩张、色素沉着或脱失、萎缩(皮肤异色症).(3)雷诺氏现象.(4)关节痛.2.检查所见:(1)肌肉活检;能证实肌纤维变性和炎症性反应(间质或血管周围细胞浸润)、结缔组织增生.(2)血清肌酸-磷酸激酶活性上升.(3)     

伴有脊柱侧弯的先天性毛细血管扩张性大理石样皮肤

报告1例罕见的皮损持续存在且伴有脊柱侧弯的先天性毛细血管扩张性大理石样皮肤。患者女,19岁。背部树枝状青紫色毛细血管扩张伴脊柱侧弯19年。皮肤科检查:背部见网状或树枝状青紫色毛细血管扩张,伴局部坏死破溃、结痂、色素脱失及萎缩凹陷。皮损组织病理:表皮轻度萎缩,棘层色素增加,表皮突向下延伸,真皮全层胶原致密、均质化,大量毛细血管扩张,血管内皮细胞增生,管周有少量淋巴细胞浸润。诊断为先天性毛细血管扩张性大理石样皮肤(cutis marmorata telangiectatica congenita, CMTC)。     

Civatte皮肤异色病1例

患者女,50岁。面部皮肤褐色斑片40余年。查体见面颈部弥漫分布黑褐色斑片,两颊呈网状分布,伴有白色斑点、毛细血管扩张和轻度萎缩。皮肤组织病理示:表皮变薄,基底细胞液化变性,真皮浅层血管周围炎细胞浸润,见明显噬色素细胞。诊断:Civatte皮肤异色病。     

膀胱粘膜损害剧痛为特征的大疱性表皮松解症一例

患者男,17岁.4岁发病.病初于会阴部皮肤出现水疱,伴有排尿痛,当时疑似膀胧结石,经用中药治疗,排尿痛似有缓解.之后不久,皮肤、口腔常有水疱,愈后残留萎缩疤.13岁时,排尿痛加重,同时伴有尿液混浊,时而呈粉红色或混有膜样物,经中药治疗未见缓解.16岁时排尿痛更为明显,故于1981年1月6日住院治疗.     

Becker痣综合征1例

正1病历摘要患者女,21岁。因左胸部皮肤萎缩、变硬伴左侧乳房发育不全11年,于2015年8月24日至我院皮肤科就诊。患者11年前左侧胸部出现大片皮肤萎缩、变厚,质地稍硬,并伴有色素沉着,皮损处皮肤可提起。随着年龄增长皮损范围缓慢扩大,自觉无不适症状。左侧乳房不发育,右侧乳房发育正常。患者既往体健,无药物及食物过敏史。否认遗传病及传染病,家族成员中无类     

角膜炎-鱼鳞病-耳聋综合征

报告1例角膜炎-鱼鳞病-耳聋综合征.患者女,21岁,全身皮肤干燥、红斑、脱屑、折皱部位皮肤增厚21年,角膜病变18年,听力下降15年.体格检查:皮肤干燥,脱屑,毛发脱落,无汗,皮肤高度角化.角膜萎缩伴有新生血管,双耳高度感音性耳聋,听力下降,皮损组织病理检查示鱼鳞病样改变.根据典型的临床表现和组织病理改变诊断为角膜炎-鱼鳞病-耳聋综合征.治疗采用阿维A 30mg/d口服.     

Occupational allergic contact dermatitis caused by decorative plants

12 cases of occupational allergic contact dermatitis caused by decorative plants were diagnosed in a 14-year period. The patients were middle-aged, and their average exposure time was 13 years. The plant families and plants causing occupational contact dermatitis were Compositae (5 patients: chrysanthemum, elecampane, gerbera, feverfew), Alstroemeriaceae (5 patients, Alstroemeria ), Liliaceae (4 patients; tulip, hyacinth). Amaryllidaceae (2 patients: narcissus) and Caryophyllaceae (2 patients; carnation, cauzeflower). The known chemical allergens causing dermatitis were tuliposide-A and sesquiterepene lactones, such as alantolactones and parthenolide, in the Liliaceae and Compositae families. 7 of the 12 patients were able to continue their work; 5 were not because of severe relapses of skin symptoms. The plant allergen and extract series currently available are of great help in the diagnosis.     

Deutliche Einschränkung der Lebensqualität bei Patienten mit Pemphigus vulgaris: Ergebnisse der deutschen Bullous Skin Disease (BSD)‐Studiengruppe

Background: Pemphigus vulgaris is a potentially life‐threatening autoimmune disorder of the skin and mucous membranes characterized by antibodies against epidermal adhesion molecules. Clinically characteristic are painful chronic blisters or erosions of mucous membranes and skin. There are no published studies on the impact o this disease on quality of life. Patients and methods: This registration was performed within the scope of the German BSD (Bullous Skin Disease) study group, from November 1997 until January 2002. A total of 36 patients with the first diagnosis of pemphigus vulgaris were registered at the university hospitals of Dresden, Erlangen, Kiel, Mannheim, München and Würzburg. Thirty of the 36 (83 %) patients participated in the quality of life questionnaire utilizing the German version of ‘Dermatology Life Quality Index’ (DLQI) provided by A. Y. Finlay. The DLQI varies from 0 to 30 with an increased DLQI score indicating a decrease in quality of quality. Results: The overall DLQI total score of 10 ± 6,7 in the investigated pemphigus patients was significantly increased in comparison to other skin diseases. Conclusions: These results suggest that the DLQI can be a very useful additional outcome criteria for clinical studies with pemphigus vulgaris and in the treatment of these patients.     

Metástasis cutáneas de tumores sólidos. Estudio descriptivo retrospectivo

Introduction

Cutaneous metastases are a rare event compared with other metastases. Their incidence is estimated between 0.7% and 9% of patients with cancer. Their presence always indicates a disseminated malignant disease and could also be the first manifestation of a tumor, appearing simultaneously or after a diagnosis of cancer.

Objectives

To describe the epidemiological and clinical characteristics of cutaneous metastases in our hospital.

Material and method

We performed an observational retrospective study. We included all patients with cutaneous metastases diagnosed in the Dermatology Service of our hospital during a 7-year period. Patients with metastases from sarcomas, melanomas and hematologic malignances were excluded.

Results

We studied 102 patients with cutaneous metastases. The primary tumor was identified in 87 patients (85%). The most frequent tumors were breast cancer in women (48%) and lung cancer in men (11%). Cutaneous metastases were the first sign of malignancy in 12 patients (11%). The predominant clinical form was the nodular form. The most common localization of the metastases was the thoracic region. The most common histologic diagnosis was adenocarcinoma. The mean survival after the development of metastases was 22.1 months.

Conclusion

There is an association between the frequency of cutaneous metastases and the most frequent malignancies in each sex.Our results were similar to those of other studies regarding age, sex distribution, the predominant clinical form, location, and histological diagnosis of cutaneous metastases.     

Mutations in mevalonate pathway genes in patients with familial or sporadic porokeratosis

Porokeratosis comprises heterogeneous keratinization disorders that are characterized by one or more atrophic patches surrounded by a ridge‐like cornoid lamella. In this study, we evaluated seven families affected by porokeratosis and five sporadic patients of the disease in a Chinese population. We performed Sanger sequencing of exons and flanking intron–exon boundaries of mevalonate pathway genes (MVD, MVK, PMVK and FDPS) and of SLC17A9. In five familial and three sporadic patients, we detected six variations, including four novel mutations (MVD c.1A>G; p.Met1?, c.916G>A; p.Ala306Thr, c.1013+1G>A, and PMVK c.65A>G; p.Lys22Arg) and two recurrent mutations (MVD c.746T>C; p.Phe249Ser, and MVK c.1028T>C; p.Leu343Pro). We then applied I‐TASSER and iGEMDOCK to assess these variants for probable functional impacts. The findings of this study extend the mutation spectrum of porokeratosis and provide further evidence for the genetic basis of this disease.     

[Translated article] Pruritus in Dermatology: Part 1—General Concepts and Pruritogens

Pruritus is the most common symptom of dermatologic and systemic diseases. The diagnosis of pruritus is clinical, although additional tests may be necessary to identify or confirm the cause. Translational medicine has led to the discovery of new mediators of itch, or pruritogens, as well as new receptors. Knowing how to properly recognize the main pathway that mediates itch in each patient is the key to successful treatment. Although the histaminergic pathway predominates in conditions like urticaria or drug-induced pruritus, it is the nonhistaminergic pathway that predominates in nearly all other skin diseases covered in this review. Part 1 of this 2-part review discusses the classification of pruritus, additional testing, the pathophysiology of itch and the pruritogens implicated (including cytokines and other molecules), and central sensitization to itch.     

Anästhesieverfahren in der Dermatologie

Zusammenfassung Die Verfahren der Lokalanästhesie sind integraler Bestandteil der operativen Dermatologie. Sie gewährleisten eine effiziente und sichere Analgesie in umschriebenen Haut- und Weichteilregionen und ermöglichen, einen sonst schmerzhaften diagnostischen oder therapeutischen Eingriff bei erhaltenem Bewusstsein zu tolerieren. Einzelne Methoden der Applikation sind "konkurrenzlos", wie die topische Applikation von EMLA^® oder die Kryoanästhesie, andere bieten alternative Optionen zur Allgemeinanästhesie. Die Tumeszenzlokalanästhesie wurde—jenseits der kosmetischen Liposuktion—zu einer effizienten Anästhesieform für größere Operationen bei Tumoren der Haut, plastische Rekonstruktionen und in der Phlebochirurgie weiterentwickelt. Die Wahl des Verfahrens im Einzelfall wird vom Alter, der Kooperationsfähigkeit und der Komorbidität des Patienten bestimmt. Für Infiltrationsanästhesien werden heute vorwiegend Lokalanästhetika vom Amidtyp eingesetzt. Fundierte Kenntnisse über die Anatomie der sensiblen Nerven sind Voraussetzung für erfolgreiche operationsfeldnahe periphere Blockaden. Wenn die Wirkungsweise der Lokalanästhetika, ihre toxischen Effekte und potenzielle Arzneimittelinteraktionen bei ihrem Metabolismus in der Praxis beachtet werden, dann ist das Risiko von Komplikationen relativ gering. Es sollte dennoch nicht unterschätzt, und adäquate Notfallmaßnahmen im Operationsteam sollten regelmäßig trainiert werden.     

Skin microbiome and acne vulgaris: Staphylococcus,a new actor in acne

Propionibacterium acnes (P. acnes), the sebaceous gland and follicular keratinocytes are considered the three actors involved in the development of acne. This exploratory study investigated the characteristics of the skin microbiota in subjects with acne and determined microbiota changes after 28 days of application of erythromycin 4% or a dermocosmetic. Skin microbiota were collected under axenic conditions from comedones, papulo‐pustular lesions and non‐lesional skin areas from subjects with mild to moderate acne according to the GEA grading using swabs. Samples were characterized using a high‐throughput sequencing approach that targets a portion of the bacterial 16S rRNA gene. Overall, microbiota samples from 26 subjects showed an overabundance of Proteobacteria and Firmicutes and an under‐representation of Actinobacteria. Staphylococci were more abundant on the surface of comedones, papules and pustules (P=.004 and P=.003 respectively) than on non‐lesional skin. Their proportions increased significantly with acne severity (P<.05 between GEA‐2 and GEA‐3). Propionibacteria represented less than 2% of the bacteria on the skin surface. At Day 28, only the number of Actinobacteria had decreased with erythromycin while the dermocosmetic decreased also the number of Staphylococci. A significant reduction (P<.05) from Day 0 of comedones, papules and pustules with no significant difference between the products was observed. The bacterial diversity on all sampling areas was similar. The dermocosmetic decreased the number of Actinobacteria and Staphylococcus spp. after 28 days. Staphylococcus remained the predominant genus of the superficial skin microbiota. No significant reduction in Staphylococcus spp. was observed with the topical antibiotic.