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引用次数: 0
摘要
局部硬皮病(LS)是一种与皮肤和皮下组织萎缩有关的炎症性硬皮病。根据亚型、病情严重程度和病变局部的不同,病变过程中会累及脂肪组织、肌肉、关节和骨骼,但不会累及内脏器官。药物在儿童期的年原发性发病率为每 100 万儿童中有 3.4 例;女性的发病率是男性的 2.6-6 倍。该病的临床表现多种多样,因此出现了大量不同的分类方法,这些方法考虑到了纤维化过程的严重程度、普遍性和深度,因此将 LS 的临床形式主要分为五种:局限型、全身型、线型、深度型和混合型。这些药物形式并不相互排斥,因为同一患者可能会出现不同的疾病表现。线状型是儿童期最常见的 LS 病型之一,约有 40-70% 的儿童患有这种病。
MODERN TREATMENT AND CORRECTION OF THE CONSEQUENCES OF LOCALIZED SCLERODERMA
Localized scleroderma (LS) is an inflammatory sclerosing disease of the skin and subcutaneous tissues associated with their atrophy. Depending on the subtype, severity of the disease and localization of the lesion, involvement in the pathological process of adipose tissue, muscles, joints and bones, but not internal organs, is noted. The annual primary incidence of drugs in childhood is 3.4 cases per 1,000,000 child population; in females, the disease occurs 2.6–6 times more often. The wide range of clinical manifestations of the disease has led to the emergence of a large number of different classifications, which take into account the severity, prevalence and depth of the fibrosis process, as a result of which five main clinical forms of LS are distinguished: limited, generalized, linear, deep and mixed. The forms of drugs are not mutually exclusive, since the same patient may experience different manifestations of the disease. One of the most common forms of LS in childhood is the linear form, which is observed in approximately 40–70% of children.