Ashworth M T, Nash J R, Ellis A, Day D W
University Department of Pathology, Liverpool, UK.
Histopathology. 1991 Oct;19(4):303-10. doi: 10.1111/j.1365-2559.1991.tb00044.x.
Tylosis is an autosomal dominant inherited defect of keratinization, associated in two Liverpool families with a high risk of developing oesophageal squamous carcinoma. In 29 individuals, followed by regular endoscopy and biopsy, we have noted several morphological abnormalities of the epithelium in this pre-cancerous condition. A control group of 43 non-tylotic patients with normal oesophageal histology and a further 26 patients with acute oesophagitis was used for comparison. Recognizable dysplasia was confined to the older age range in the tylotic group and was present in four patients. Almost half of the patients showed acute inflammation. Abnormalities of maturation were common, the most frequent being the presence of prominent basophilic inclusions and clear cell acanthosis, with parakeratosis and frank surface keratinization present in smaller numbers. There was, however, no statistically significant difference between the tylotic and inflamed control groups for any of these features. The only feature to show a significant difference between these groups was the presence of individual cell keratinization. The results suggest that in the oesophageal epithelium of the patients with tylosis, inflammation is the predominant abnormality, together with individual cell keratinization, and that these lesions appear in a much younger age group than dysplasia.
掌跖角化病是一种常染色体显性遗传的角化缺陷病，在利物浦的两个家族中，其与发生食管鳞状癌的高风险相关。在29名接受定期内镜检查和活检的个体中，我们注意到在这种癌前状态下上皮存在几种形态学异常。选取43名食管组织学正常的非掌跖角化病患者作为对照组，并选取另外26名急性食管炎患者用于比较。可识别的发育异常局限于掌跖角化病组的老年人群，有4名患者存在该情况。几乎一半的患者表现出急性炎症。成熟异常很常见，最常见的是出现明显的嗜碱性包涵体和透明细胞棘皮症，较少见的是不全角化和明显的表面角化。然而，对于这些特征中的任何一项，掌跖角化病组与炎症对照组之间均无统计学上的显著差异。两组之间唯一显示出显著差异的特征是单个细胞角化的存在。结果表明，在掌跖角化病患者的食管上皮中，炎症是主要异常，同时伴有单个细胞角化，并且这些病变出现在比发育异常年轻得多的年龄组中。
