Maillefer R H, Greydanus M P
Blodgett Memorial Medical Center, Grand Rapids, Michigan, USA.
Am J Gastroenterol. 1999 Mar;94(3):829-34. doi: 10.1111/j.1572-0241.1999.00954.x.
Tylosis is a rare, autosomal dominant syndrome presenting with hyperkeratosis of the palms and soles of the feet. Two types have been identified. Late onset tylosis (type A) is reported to be associated with a high incidence of esophageal carcinoma, whereas early onset tylosis (type B) appears to be a benign disorder. This distinction has significant implications for surveillance and prognosis. We report two families exhibiting early onset type B tylosis, spanning five and seven generations, respectively, and believe these to be the first two extensive genealogies of tylosis type B reported in North America. They serve to verify the features of type B tylosis and its benign prognosis. The world literature is reviewed and clinical relevance is discussed. Recommendations for follow-up of afflicted individuals are proposed.
掌跖角化病是一种罕见的常染色体显性综合征，表现为手掌和脚底的角化过度。已确定有两种类型。迟发性掌跖角化病（A型）据报道与食管癌的高发病率相关，而早发性掌跖角化病（B型）似乎是一种良性疾病。这种区分对监测和预后具有重要意义。我们报告了两个分别跨越五代和七代的早发性B型掌跖角化病家族，并认为这是北美报道的前两个B型掌跖角化病的大型家系。它们有助于验证B型掌跖角化病的特征及其良性预后。我们回顾了世界文献并讨论了临床相关性。提出了对患病个体进行随访的建议。
