Proceeding report of the Second Symposium on Hidradenitis Suppurativa Advances (SHSA) 2017

The 2nd Annual Symposium on Hidradenitis Suppurativa Advances (SHSA) took place on 03‐05 November 2017 in Detroit, Michigan, USA. This symposium was a joint meeting of the Hidradenitis Suppurativa Foundation (HSF Inc.) founded in the USA, and the Canadian Hidradenitis Suppurativa Foundation (CHSF). This was the second annual meeting of the SHSA with experts from different disciplines arriving from North America, Europe and Australia, in a joint aim to discuss most recent innovations, practical challenges and potential solutions to issues related in the management and care of Hidradenitis Suppurativa patients. The last session involved clinicians, patients and their families in an effort to educate them more about the disease.     

Prevalence of Acne Inversa (Hidradenitis Suppurativa) in China: A Nationwide Cross-Sectional Epidemiological Study

Objective::This study was performed to estimate the prevalence of hidradenitis suppurativa (HS) in China and describe the demographic and clinical features of H...     

Integrating complement into the molecular pathogenesis of Hidradenitis Suppurativa

Complement inhibition has been identified as a potential therapeutic target for multiple inflammatory disorders including Hidradenitis Suppurativa (HS). It is currently unclear how complement integrates into our current model of molecular pathogenesis in HS and whether it represents a central component of pathogenesis, or a neutrophil-associated bystander. Levels of C5a in serum and tissue correlate with disease activity and degree of neutrophilic infiltrates in HS. C5a has been associated with Th17 immune axis activation in psoriasis, rheumatoid arthritis and Crohn's disease with strong similarities to TH17 activation in HS. Porphyromonas species (which are identified in the HS microbiome) are able to cleave inactive C5 into C5a implicating the cutaneous microbiome as an activator of complement. C3a and C5a are associated with activation of the NLRP3 inflammasome, implicated in the inflammatory drive in HS. Complement receptors are present upon dendritic cells, monocytes, fibroblasts and adipocytes, which may broaden the potential contribution of complement to multiple aspects of HS pathogenesis. Dysregulation of complement receptor pathways has been documented in obesity, insulin resistance and polycystic ovarian syndrome leading to the possibility that complement may explain the epidemiological associations between these conditions and HS. The therapeutic potential of complement inhibitors in HS may be related to the therapeutic target (complement receptor or complement subunit) and the presence of alternate receptors (such as C5aR2) or ligands (including C3a, PAMPs and DAMPs). Integrating complement into the known pathogenesis of HS may aid in explaining the contradictory results between Phase 2 studies of C5a antagonists. It also allows for the identification of existing knowledge gaps to target further clinical investigation and research.     

Netherton综合征研究进展

 Netherton综合征（NS）是由SPINK5基因突变引起的一种罕见隐性遗传性鱼鳞病,其特征是鱼鳞病样红皮病（CIE）和/或迂回线状鱼鳞病（ILC）、毛干异常和IgE水平升高三联症。因三联征并不总是完全存在,NS患者常常面临误诊、治疗延迟,甚至治疗无效的难题。本文全面回顾了NS的发病机制进展、临床特征、组织病理学和免疫组织化学、诊断和鉴别诊断以及治疗进展,以达到早期识别、有效干预NS的目的。     

原发皮肤恶性黑色素瘤细胞分子遗传学研究进展

本文综述了原发皮肤恶性黑色素细胞分子遗传学改变，染色体上的非随机改变主要发生在1、6、9、7及10号染色体上。     

下肢肌周细胞瘤一例

患者,女,33岁。因右小腿皮肤丘疹30余年,伴红肿疼痛3个月。予以手术切除,术后病理示：瘤细胞以多层同心圆型围绕薄壁血管周围生长,部分可见血管管腔闭塞。免疫组化示：ERG(血管+),CD34(血管+),CD31(血管+),SMA(+),Desmin(-),H-caldesmon(+)。结合形态学及免疫组化考虑肌周细胞瘤。予以手术扩大切除后植皮。     

Pain and Late-Onset of Hidradenitis Suppurativa Can Have a Negative Influence on Occupational Status and Educational Level. A Cross-Sectional Study

Background and objectivesHidradenitis suppurativa is a chronic and painful condition with negative impact on daily activity. Little information on the impact of disease-specific factors on educational level and occupational status in hidradenitis suppurativa patients has been reported. We sought to identify how disease-specific factors could influence occupational status and educational level in patients with hidradenitis suppurativa.MethodsCross-sectional study of patients with hidradenitis suppurativa seen between September 2017 and September 2018. Disease-specific variables were analyzed to find associations in patients with different educational levels and occupational status.ResultsNinety-eight patients were included. Patients with non-university studies had more frequently ≥ 3 affected areas (22.5% [16/73] vs 4.8% [1/22], p = 0.049), a higher number of painful days (8.5 [SD 8.8] vs 4.6 [SD 4.8], p = 0.048) and a higher score on the VAS scale (6.7 [SD 2.8] vs 5.0 [3.3], p = 0.031). Patients from the inactive group had a significantly increased number of painful days (11.2 [SD 10.4] vs 5.7 [SD 6.2], p = 0.004). This group had a greater number of patients with a history of depression (61.3% [19/31] vs 27.4% [17/62], p = 0.002) and a higher mean BMI (32.3 [9.1] vs 28.4 [6.4], p = 0.016). Late disease onset was significantly associated with being “inactive” (26.7% [8/31] vs 6.5% [4/62], p = 0.026). No significant differences between severity scales of hidradenitis suppurativa and educational level or occupational status were found. Limitations: cross-sectional and single center study.ConclusionsPain, ≥3 affected areas, history of depression, higher mean BMI, and late onset of hidradenitis suppurativa, are associated with low education level and inactive occupational status.     

Prevalence of the MspI and Ile462Val SNPs of Cytochrome P‐450 1A1 in Hidradenitis Suppurativa

Please cite this paper as: Prevalence of the MspI and Ile⁴⁶²Val SNPs of Cytochrome P‐450 1A1 in Hidradenitis Suppurativa. Experimental Dermatology 2010; 19: 541–542. Abstract: Hidradenitis Suppurativa (HS) is a chronic inflammatory skin disease that affects the hair follicles in the axillary, perianal and inguinal area. Its cause and pathogenesis are unknown, but cigarette smoking increases the risk of developing HS conceivably by accumulating toxic metabolites in sweat. The xenobiotic compounds from tobacco are metabolized by the cytochromes P‐450. The cytochrome P‐450 1A1 (CYP1A1), one of the most active isoenzymes, harbours several polymorphisms. Two of them, MspI and Ile⁴⁶²Val single nucleotide polymorphism (SNP), are associated with enhanced activity and inducibility. Performing direct DNA sequencing, we investigated the frequencies of these SNP in 51 patients with HS, 45 of these were smokers. We found similar overall SNP rates in our patients in comparison with previous data for Caucasian or German controls. Obviously, there is no relation between the occurrence of these SNPs and the risk of developing HS.     

痤疮的发病机制研究进展

痤疮的病因及发病机理较为复杂，性激素分泌及对皮脂腺的调控异常、皮脂腺角化异常、毛囊内微生物、炎症损害及免疫反应，另外遗传因素、心理因素等均参与痤疮的整个发病过程。     

Poikiloderma of Civatte: a clinical and epidemiological study

BACKGROUND: Although a common dermatosis, idiopathic poikiloderma of the face and neck has not been studied in depth for decades. OBJECTIVES: To reassess the clinical and epidemiological characteristics of poikiloderma of Civatte (PC). MATERIAL AND METHODS: Fifty consecutive patients with PC. Evaluation included history taking and physical examination. Epidemiological and clinical parameters were recorded and analysed. The literature from 1923 until today, was reviewed thoroughly. RESULTS: The frequency of PC among dermatologic patients was estimated to be 1.4%. There were 34 females (68%) and 16 males in the present study. The mean age at diagnosis was 47.8 years for females and 61.7 years for males. The majority (88%) had skin phototype II or III. Among females, 26 were at their peri-menopausal stage, including three cases of iatrogenic menopause. Four patients reported that other blood-related family members also had PC. The v and the sides of the neck and the upper chest were most often affected in a symmetric distribution. The face (preauricular and parotid region) was involved in 19 patients (38%). The erythemato-telangiectatic clinical type predominated (58%), followed by the mixed (22%) and the pigmented type (20%). Almost half of the patients (46%) were symptomatic (itching, burning and 'flushing'). The mean duration from onset to diagnosis was 6.2 years according to the patients' report. The course was usually slowly progressive (82%) and irreversible. CONCLUSIONS: PC shows characteristic features, supporting the theory that it represents a distinct entity. It is rather common in Greece. Although menopausal women predominated in our cohort, men were not uncommonly affected and were diagnosed at an older age. Based on the predominating clinical feature, PC can be classified into three clinical forms. Symmetry and sparing of the anatomically shaded areas of the neck are highly characteristic for PC. Face involvement was not as common and as severe as it had been considered in the past. Recognition of clinical type is important for the selection of the most appropriate treatment, which, despite the advent of novel modalities, remains problematic.     

2004-2018年山东省淋病流行病学分析

收集2004-2018年山东省报告的淋病病例资料,运用描述流行病学方法和SPSS 23.0软件进行分析.结果示2004-2018年山东省淋病年均报告发病率为4.19/105,山东省淋病维持在低流行水平,低于全国平均流行水平(χ2=18.434,P<0.05).淋病发病存在明显区域和人群分布差异,年均报告发病率居于前5位...     

老年特应性皮炎的诊疗研究进展

 特应性皮炎(AD)是一种与遗传有关的慢性、炎症性、瘙痒性皮肤病。随着社会发展及人口老龄化,老年AD作为AD的一种新临床亚型出现在大家的视野并且受到广泛的关注。不同于其他三型AD（婴儿期、儿童期、成人期）,老年人常患有临床表现相似的其他瘙痒性疾病（如脂溢性皮炎、老年瘙痒症、大疱性类天疱疮等）并且伴有其他潜在病症（如高血压病、心脑血管疾病、糖尿病等）,因此老年AD的诊断与治疗较其他三型复杂。本文主要对老年AD的流行病学、临床特征、诊断、鉴别诊断、治疗与管理进行综述,以引起临床医生对该新临床亚型的重视以及为老年AD的诊断与治疗提供思路。