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首页> 外文期刊>Clinics in dermatology >The immunocompromised district in dermatology: A unifying pathogenic view of the regional immune dysregulation
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The immunocompromised district in dermatology: A unifying pathogenic view of the regional immune dysregulation

机译:皮肤科免疫功能低下的地区:区域免疫失调的统一病原学观点

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摘要

Besides the systemic immune deficiency, a sectorial default in immune control may occur in immunocompetent subjects. This regional immune defect can appear and remain confined to differently damaged skin areas, lately labeled immunocompromised districts (ICDs).An ICD is a skin area more vulnerable than the rest of the body for genetic or acquired reasons. Its vulnerability mainly consists in a local dysregulation of the immune control, which often facilitates (but sometimes hinders) the local onset of immunity-related eruptions or skin disorders.The factors responsible for localized immune dysregulation are multifarious, being represented by chronic lymphatic stasis, herpetic infections, ionizing or ultraviolet (UV) radiations, burns, all sorts of trauma (especially amputation), tattooing, intradermal vaccinations, and others of disparate nature (eg, paralytic stroke, poliomyelitis). Whatever the cause, in time an ICD may become a vulnerable site, prone to developing opportunistic infections, tumors, or dysimmune reactions (often of granulomatous type), strictly confined to the district itself; however, the opposite may also occur with systemic immune disorders or malignancies that selectively spare the district. In any case, the immunologic behavior of an ICD is different from that of the rest of the body. The pathomechanisms involved in this sectorial immune destabilization may reside in locally hampered lymph drainage that hinders the normal trafficking of immunocompetent cells (eg, chronic lymphedema, posttraumatic lymph stasis) or in a damage to sensory nerve fibers that release immunity-related peptides (eg, herpetic infections, carpal tunnel syndrome), or in both conditions (eg, amputation stump, radiation dermatitis).The ICD is a conceptual entity with no definite shape or dimension. It may take an extremely variable form and extent depending on the causative agent, ranging from a minimal area (eg, intradermal vaccination) or a small area (eg, herpes simplex infection), through a wide area (eg, radiotherapy), a bandlike segment (eg, skin mosaicism, herpes zoster infection), or an acral area (eg, carpal tunnel syndrome), up to a whole limb (eg, Stewart-Treves syndrome) or even an entire half body (eg, brain stroke).Varied newly coined terminology can be used to indicate the specific cause each time that it is responsible for a regional immune dysregulation. The advantage of the umbrella term ICD is that it encompasses all the possible causes involved in a local immune destabilization.An ICD may have a congenital or a postnatal origin, and interesting similarities between the two forms exist. An ICD may also take place in patients with a preexisting systemic immune deficiency, thus creating a more vulnerable site in an already vulnerable patient.Identifying a cutaneous ICD in a given patient is an important standpoint for both diagnostic and prevention purposes. This can be proven by the educative clinical examples that are reported here.
机译:除全身性免疫缺陷外,免疫功能正常的受试者可能会出现免疫控制方面的部门默认。这种区域性免疫缺陷可能会出现并保持局限在受损程度不同的皮肤区域,即最近标记的免疫功能低下的区域(ICD)。由于遗传或后天原因,ICD是比身体其他部位更脆弱的皮肤区域。它的脆弱性主要在于免疫控制的局部失调,通常促进(但有时阻碍)与免疫有关的爆发或皮肤疾病的局部发作。造成局部免疫失调的因素多种多样,以慢性淋巴淤滞为代表,疱疹感染,电离或紫外线(UV)辐射,烧伤,各种创伤(尤其是截肢),纹身,皮内疫苗接种以及其他性质不同的疾病(例如麻痹性中风,小儿麻痹症)。不管是什么原因,随着时间的流逝,ICD可能会成为易受感染的部位,容易发展为机会性感染,肿瘤或免疫反应不良(通常是肉芽肿型),严格限于该地区本身;但是,对于全身免疫性疾病或恶性肿瘤,也有可能出现相反的情况,这些疾病或恶性肿瘤会选择性地使该地区免身。在任何情况下,ICD的免疫学行为都与身体其他部位不同。涉及该部门免疫失稳的病理机制可能存在于局部受阻的淋巴引流中,这阻碍了免疫功能细胞的正常运输(例如,慢性淋巴水肿,创伤后淋巴淤滞)或损害了释放免疫相关肽的感觉神经纤维(例如,疱疹感染,腕管综合症)或两种情况(例如截肢残端,放射性皮炎)。ICD是概念性实体,没有明确的形状或尺寸。根据病原体的不同,其形式和程度可能会变化很大,范围从最小区域(例如,皮内接种疫苗)或小区域(例如,单纯疱疹感染)到大面积(例如,放射疗法),带状部分(例如,皮肤镶嵌症,带状疱疹感染)或肢端区域(例如,腕管综合症),直至整个肢体(例如,Stewart-Treves综合征)或整个半身(例如,脑中风)。每次使用新产生的各种术语来表示引起区域免疫失调的具体原因时,都可以使用该术语。总体术语ICD的优势在于它涵盖了涉及局部免疫不稳定的所有可能原因.ICD可能具有先天性或出生后起源,并且两种形式之间存在有趣的相似性。患有先天性全身免疫缺陷的患者也可能发生ICD,从而在本来就很脆弱的患者中形成一个更易受伤害的部位。识别特定患者的皮肤ICD是诊断和预防目的的重要观点。这可以通过此处报告的教育性临床实例证明。

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