中华皮肤科杂志 ›› 2010, Vol. 43 ›› Issue (4): 289-289.

• 病例报告 • 上一篇    下一篇

痣样基底细胞癌综合征一例

王桂芝1,潘敏2,史同新3,王君1   

  1. 1. 青岛大学医学院附属医院皮肤科
    2. 青岛大学医学院附属医院
    3. 青岛大学医学院附属医院性健康中心
  • 收稿日期:2009-06-15 修回日期:2009-12-01 出版日期:2010-04-15 发布日期:2010-04-07
  • 通讯作者: 王桂芝 E-mail:wangguizhi1956@163.com

A case of nevoid basal cell carcinoma syndrome

  • Received:2009-06-15 Revised:2009-12-01 Online:2010-04-15 Published:2010-04-07

摘要:

痣样基底细胞癌综合征是一种罕见的常染色体显性遗传病,以泛发性皮肤基底细胞癌和多器官发育异常为主要临床特征。本文报告1例痣样基底细胞癌综合征患者,并结合相关文献对该病的发病率、发病机制、诊断标准、治疗方法等进行讨论。

关键词: 痣样基底细胞癌综合征, 基底细胞癌

Abstract:

[Abstract] Objective To report a case of nevoid basal cell carcinoma syndrome and discuss its incidence, pathogenesis, clinical features and treatment. Methods Skin specimens were obtained from the patient and examined by histopathology , and relevant literatures were reviewed. Results A 48-year-old female presented with a 40-year history of with decades of crusted macules, varying in color from pink to brown and ranging in size from 0.5 to 3 mm in diameter, together with palmar keratotic plaques. Histopathology of skin specimens on the chest, abdomen and back revealed the presence of nests and lobules of basophile cells orderly settled, with a great amount of melanin pigmentation. The patient is mending after 3-month treatment with oral retinoid therapy . Conclusions The case is diagnosed as nevoid basal cell carcinoma syndrome by clinical features and histopathology findings, and was effectively treated by retinoic acid.

Key words: nevoid basal cell carcinoma syndrome, basal cell carcinoma