Darier病的典型表现：一种罕见的角化异常疾病。

Classical presentation of Darier's disease: a rare disorder of keratinisation.

作者信息

Mahboob Atiya, Yaqub Faryal, Shahzad Zahid, Afzaal Manaal, Hanif Moazzum, Khan Deeba Sattar, Nasim Riffat, Khan Aleena

机构信息

Department of Dermatology, Shaikh Zayed Federal Postgraduate Medical Institute, Lahore, Pakistan.

出版信息

J Ayub Med Coll Abbottabad. 2010 Jul-Sep;22(3):230-3.

PMID:22338464

Abstract

Keratosis follicularis or Darier's disease (DD), a rare autosomal dominant disorder is characterised clinically by appearance of multiple, prurutic, discrete, scaly papules affecting seborrheic areas coupled with palmar pits, nail changes and mucosal involvement. Histologicaly the lesions show suprabasal clefts with acantholytic and dyskeratotic cells. We report a case of 35 years old woman with typical clinical and histological features of Darier's disease.

摘要

毛囊角化病或达里埃病（DD）是一种罕见的常染色体显性疾病，临床上的特征是出现多个瘙痒性、离散性、鳞屑性丘疹，累及脂溢性区域，并伴有掌跖凹陷、指甲改变和黏膜受累。组织学上，病变表现为基底层上的裂隙，伴有棘层松解和角化不良细胞。我们报告一例35岁女性患者，具有典型的达里埃病临床和组织学特征。