Granulomatous hidradenitis suppurativa and cutaneous Crohn's disease

Three patients with concurrent hidradenitis suppurativa and Crohn's disease are presented. The notable histological feature in each hidradenitis resection was the presence of numerous discrete epithelioid granulomas in areas of non-inflamed dermis. The purpose of the study was to determine the incidence of epithelioid granulomas in 101 hidradenitis patients and their significance in relation to systemic granulomatous disease. Discrete epithelioid granulomas were identified in 8% of the resections (10 patients). One patient had Crohn's disease and one other pulmonary sarcoidosis. Seven patients with granulomatous hidradenitis neither had nor developed any other disease during the 3-year follow-up period. Clinical review identified a further two patients with Crohn's disease but associated with non-granulomatous changes in the skin resections. Foreign body type granulomas were identified in 25% of the resections adjacent to ruptured hair follicles, sinus tracts or nearby degenerate sweat glands. The study shows that, although foreign body type granulomas are a common finding in hidradenitis, the presence of discrete epithelioid granulomas in the dermis away from the site of active inflammation is unusual and should alert the pathologist to the possibility of systemic granulomatous disease such as Crohn's disease or sarcoidosis.     

Reconstruction of the male external genitalia in diverse disease processes: Our reconstructive algorithm,techniques, and experience

BackgroundMale genital form and function may be rendered abnormal by a number of disease processes, with profound associated psychological and functional consequences. The aim of the study is to review our reconstructive experience with cases of genital loss or distortion due to nonmalignant diseases processes and atypical neoplasia.Materials and methodsA retrospective review of a prospectively maintained database was performed to identify reconstructive cases performed from 2018 to 2020 under the care of a single surgeon. Male patients 18 years or older with a disease diagnosis other than squamous cell carcinoma affecting genital form were included. Disease processes, patient factors, surgical techniques, and both functional and cosmetic outcomes were reviewed.ResultsFourteen cases were identified. The patients had a mean age of 52.2 years (range, 21–72 years). Acquired buried penis was present in 8 patients. Etiology of genital abnormality included balanitis xerotica obliterans (n = 6), excess skin loss at circumcision (n = 2), self-injection of petroleum jelly to penile shaft (n = 1), Fournier gangrene (n = 1), hidradenitis suppurativa (n = 1), extramammary Paget disease (n = 1), idiopathic lymphoedema (n = 1), and penoscrotal webbing (n = 1). Reconstructive techniques performed included penile debridement/shaft skin release, scrotectomy, suprapubic apronectomy, and division of penoscrotal webbing, in combination with split-thickness skin grafting where required. A penile implant was inserted in one patient. Reconstructive planning, techniques, and outcomes are described.ConclusionsA variety of reconstructive techniques in andrology can be used to improve the aesthetic and functional outcomes of multiple disease processes affecting the male external genitalia.     

Magnetic Resonance Imaging of Hidradenitis Suppurativa: A Focus on the Anoperineal Location

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease involving apocrine-bearing sites. It is characterized by recurrent painful nodules and abscesses that potentially rupture, resulting in sinus tract formation, fistulas, and scarring. HS tends to be found in the intertriginous areas (i.e., the axillary, inguinal, and perianal areas of the body). HS may be uncommon for radiologists because its diagnosis is usually based on clinical assessment. However, diagnosis based solely on clinical manifestations can underestimate the severity of HS. Ultrasonography and MRI play a critical adjunct role in determining the severity and extent of the disease and greatly aid its management. Given that MRI is an effective imaging tool, its role in the analysis of severe and anogenital HS lesions merits considerable attention. Unfortunately, anoperineal HS imposes diagnostic dilemmas. It has multiple symptoms and presentations and often mimics other diseases in the intertriginous areas. Therefore, a thorough understanding of HS is essential to avoid delayed diagnoses. This review highlights the typical MRI imaging features and staging of HS, emphasizing on the anoperineal location. The review also differentiates the disease from mimics to facilitate the prompt delivery of appropriate treatment and improve patients’ quality of life.     

Treatment of long-standing extensive perianal hidradenitis suppurativa using double rotation plasty,V-Y plasty and free grafts

Background Perianal hidradenitis suppurativa is a chronic recurrent inflammatory, suppurating, and fistulizing disease of apocrine glands, adjacent anal canal skin, and soft tissues. The standard treatment used for extensive cases is a staged surgical procedure allowing the wound to heal by secondary intention or the delayed use of skin grafts.Case presentation A long-standing case, disabling for the patient, with extensive involvement of the buttock region, treated in one stage, which for reconstruction required the use of sliding plasties and free skin grafts, is reported.Results The outcome was satisfactory. Primary closure after wide excision using plastic-surgery techniques may help us resolve complex situations and obtain good results and a rapid recovery.Conclusion The method of closure with a combination of skin flaps and skin graft in one stage can be considered a valid surgical option for a group of patients with extensive perianal hidradenitis.     

Hydradenitis suppurativa and inflammatory bowel disease: An unusual,but existing association

Inflammatory bowel disease (IBD) could be associated with several extra-intestinal manifestations (EIMs) involving musculoskeletal, hepatopancreatobiliary, ocular, renal, and pulmonary systems, as well as the skin. In the last years, hidradenitis suppurativa (HS) is acquiring an increasing interest. IBD, especially Crohn’s disease (CD), is among the most reported associated diseases in HS patients. The aim of this paper is to give a brief overview of data showing a possible epidemiologic and pathogenetic association between IBD and HS. We performed a pooled-data analysis of four studies and pooled prevalence of HS in IBD patients was 12.8%, with a 95%CI of 11.7%-13.9%. HS was present in 17.3% of subjects with CD (95%CI: 15.5%-19.1%) and in 8.5% of UC patients (95%CI: 7.0%-9.9%). Some items, especially altered immune imbalance, are generally involved in IBD pathogenesis as well as invoked by HS. Smoking is one of the most relevant risk factors for both disorders, representing a predictor of their severity, despite, actually, there being a lack of studies analyzing a possible shared pathway. A role for inheritance in HS and CD pathogenesis has been supposed. Despite a genetic susceptibility having been demonstrated for both diseases, further studies are needed to investigate a genetic mutual route. Although the pathogenesis of IBD and HS is generally linked to alterations of the immune response, recent findings suggest a role for intestinal and skin microbiota, respectively. In detail, the frequent finding of Staphylococcus aureus and coagulase-negative staphylococci on HS cutaneous lesions suggests a bacterial involvement in disease pathogenesis. Moreover, microflora varies in the different cutaneous regions of the body and, consequently, two different profiles of HS patients have been identified on these bases. On the other hand, it is well-known that intestinal microbiota may be considered as “the explosive mixture” at the origin of IBD despite the exact relationship having not been completely clarified yet. A better comprehension of the role that some bacterial species play in the IBD pathogenesis may be essential to develop appropriate management strategies in the near future. A final point is represented by some similarities in the therapeutic management of HS and IBD, since they may be controlled by immunomodulatory drugs. In conclusion, an unregulated inflammation may cause the lesions typical of both HS and IBD, particularly when they coexist. However, this is still a largely unexplored field.     

臀部化脓性汗腺炎继发巨大鳞状细胞癌1例

报告臀部化脓性汗腺炎继发巨大皮肤鳞状细胞癌1例。患者男,62岁,左臀部结节破溃伴痛40余年,外生性肿物20余年。皮肤科检查：左臀部覆盖黑色斑疹,质地较硬,边界尚清,表面凹凸不平,中央可见数个脓肿、窦道、瘘管及瘢痕,挤压后见淡黄色脓性分泌物流出,伴明显触痛、恶臭;左臀部下方近肛门侧见一约20 cm×20 cm大小的外生性肿块,呈菜花状生长,质地坚硬,活动性差,表面溃烂,可见较多脓性分泌物覆盖,触之易出血。直肠指诊：肛门松紧度可,肛内未触及肿块,指套不带血,腹股沟淋巴结无肿大。左侧臀部外生性肿块组织病理：肿瘤位于真皮内,部分与表皮相连,由鳞状上皮细胞团块组成,部分细胞胞浆透明,部分区域棘刺松解,异型性明显。免疫组织化学染色检查：P63(+),P40(+),CK5/6(+),CK7(-)。诊断：(左臀部)皮肤鳞状细胞癌、化脓性汗腺炎。治疗：手术切除。随访1年未见复发。     

NCSTN基因沉默对HaCaT细胞增殖分化的影响

【摘要】 目的 检测NCSTN基因稳定沉默的人永生化角质形成细胞（HaCaT）增殖活性及相关分化蛋白的改变,初步探索反常性痤疮发病的可能机制。方法 构建慢病毒介导shRNA沉默NCSTN基因的HaCaT细胞模型（shRNA组）,转染空载慢病毒的HaCaT细胞作为阴性对照组,实时定量PCR和Western印迹法检测NCSTN基因的沉默效率。CCK8法检测HaCaT细胞增殖活性,实时定量PCR和Western印迹法检测HaCaT细胞角蛋白（CK1、CK5、CK7、CK10、CK14、CK16、CK17、CK18、CK19、CK20）及其他分化分子（内披蛋白、兜甲蛋白）mRNA和蛋白的表达。两组计量资料的比较采用两独立样本t检验。结果 shRNA组NCSTN mRNA及蛋白表达（0.42 ± 0.19、0.30 ± 0.07）均显著低于阴性对照组（1.00 ± 0.34、1.00 ± 0.26;t = 5.196、2.637,P < 0.001、< 0.05）,基因沉默效率达70%。与阴性对照组相比,shRNA组HaCaT细胞明显增殖活跃,CK16、CK19蛋白表达显著下调（t = 3.787、3.817,P < 0.01、< 0.05）,终末分化分子内披蛋白表达明显降低（t = 2.904,P < 0.05）。结论 稳定沉默NCSTN基因表达会引起HaCaT细胞异常增殖分化,为后续探究NCSTN基因突变引起反常性痤疮提供新思路。     

慢病毒介导shRNA沉默Nicastrin基因的人永生化角质形成细胞模型构建

目的 构建Nicastrin(NCT)基因的RNA干扰慢病毒表达载体,并在人永生化角质形成细胞(HaCaT细胞)上鉴定其沉默效率,构建NCT基因稳定下调的人永生化角质形成细胞模型,为后续研究NCT基因下调对角质形成细胞的生物学行为影响奠定实验基础.方法 设计3个靶向NCT基因特异性短发卡RNA(shRNA)表达序列并连接到慢病毒表达质粒pGLV3/H 1/GFP+ Puro中,成功构建慢病毒重组表达质粒,并通过测序鉴定.将构建的重组表达质粒与包装质粒共转染293T细胞,产生慢病毒颗粒,并测其滴度.将HaCaT细胞分为空白组(未感染病毒)、阴性对照组(NC组,感染空载病毒LV3-shNC)和干扰组(感染NCT-shRNA1、2、3慢病毒).流式细胞仪检测慢病毒转染效率,实时荧光定量PCR(qRT-PCR)、Western印迹检测靶基因的沉默效率,筛选出沉默效果最佳的干扰序列.结果 测序表明,NCT-shRNA慢病毒重组表达质粒构建成功.重组表达质粒与包装质粒共转染293T细胞后产生的慢病毒颗粒滴度为109 TU/ml.慢病毒感染HaCaT细胞后,流式细胞仪检测,慢病毒转染效率大于95％.qRT-PCR结果,与NC组相比,干扰组NCT mRNA表达量明显下降,其中NCT-shRNA1组NCT基因沉默效果最佳,干扰效率达到75％.Western印迹结果,与NC组相比,shRNA1组NCT蛋白表达抑制率为71.7％.结论 成功筛选出高效NCT-shRNA干扰序列,构建NCT-shRNA慢病毒重组表达载体,并构建NCT基因稳定下调的人永生化角质形成细胞模型.