Metze Dieter, Süßmuth Kira, Traupe Heiko
Klinik für Hautkrankheiten, Universitätsklinikum Münster, Von-Esmarch-Str. 56, 48149, Münster, Deutschland.
Pathologe. 2020 Jul;41(4):326-333. doi: 10.1007/s00292-020-00792-x.
Ichthyoses are hereditary cornification disorders that occur in isolation (nonsyndromic) or with associated internal diseases (syndromic) and can lead to life-threatening complications. The identification of the genetic causes has led to an understanding of the molecular mechanisms, but also to reclassification. The pathological changes in skin biopsies were also more precisely characterized. Certain histological patterns could be defined, which are based on the defects of epidermal differentiation but also on the inflammatory pattern. Complementary histo- and immunohistochemical methods sometimes allow a precise diagnosis, or at least a limitation of the differential diagnoses.
鱼鳞病是遗传性角化障碍疾病,可单独出现(非综合征型)或伴有相关的内科疾病(综合征型),并可导致危及生命的并发症。基因病因的确定不仅使人们对分子机制有了认识,也促使了重新分类。皮肤活检中的病理变化也得到了更精确的描述。可以定义某些基于表皮分化缺陷以及炎症模式的组织学模式。补充的组织学和免疫组化方法有时可以做出精确诊断,或者至少缩小鉴别诊断范围。