皮肤肉样瘤病是一个跨学科问题

Alexei V. Samtsov, E. Kryukov
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摘要

肉样瘤病是一种病因不明的疾病,其特征是在多个器官或组织中形成上皮样细胞肉芽肿,但无酪氨酸坏死(可能发生纤维素性坏死)。肉样瘤病几乎可累及所有器官和组织,这就决定了该问题的跨学科性质。皮肤肉样瘤病是全身性肉样瘤病最常见的表现之一。同时,皮肤科医生在肉样瘤病诊断中的作用也特别大,因为皮肤病变往往是发现全身性肉样瘤病的关键,这使得内科医生可以有目的、及时地诊断和治疗内脏器官的病变。肉样瘤病患者的皮疹可能是在肺部、胸内结节和其他内脏器官发生特殊病变的背景下继发的,但皮肤肉样瘤病也可能是主要病变。所有形式的皮肤肉样瘤病都可能是该病的唯一表现,临床和组织学上无法区分全身性肉样瘤病的皮肤变化和单纯的皮肤病变。皮肤肉样瘤病的治疗通常非常困难,根据发病率和病程的活跃程度,包括局部治疗(皮质类固醇激素、他克莫司)和全身治疗(免疫调节剂、免疫抑制剂、TNF 抑制剂)。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Sarcoidosis of the Skin as an Interdisciplinary Problem
Sarcoidosis is a disease of unknown etiology characterized by the formation of epithelioid cell granulomas in several organs or tissues without caseous necrosis (fibrinoid necrosis may occur). With sarcoidosis, almost all organs and tissues can be affected, which determines the interdisciplinary nature of the problem. Skin sarcoidosis is one of the most common manifestations of systemic sarcoidosis. At the same time, the role of dermatologists in the diagnosis of sarcoidosis is exceptionally large, since skin lesions are often the key to the discovery of systemic sarcoidosis, which allows internists to purposefully and timely diagnose and treat lesions of internal organs. Skin rashes in patients with sarcoidosis may occur secondarily against the background of specific lesions of the lungs, intrathoracic nodes and other internal organs, however, skin sarcoidosis may also develop primarily. All forms of skin sarcoidosis may be the only manifestation of the disease, and it is impossible to distinguish clinically and histologically between skin changes in systemic sarcoidosis and skin lesions alone. Treatment of skin sarcoidosis often presents great difficulties and includes, depending on the prevalence and activity of the process, local (corticosteroids, tacrolimus) and systemic therapy (immunomodulators, immunosuppressors, TNF inhibitors).
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