Baykal C, Büyükbabani N, Kavak A
Dermatology Department, Istanbul University, Istanbul Medical School.
Eur J Dermatol. 1998 Sep;8(6):385-7.
Dyskeratosis congenita is a rare, hereditary, multisystem disorder characterized by mucocutaneous changes, pancytopenia and increased incidence of malignancy. Different types of neoplasia have been reported in association with dyskeratosis congenita. We present a second case associated with Hodgkin's disease. Delayed appearance of dermatological signs and association with chronic hepatitis B are other unusual features of this case.
先天性角化不良是一种罕见的遗传性多系统疾病，其特征为皮肤黏膜改变、全血细胞减少和恶性肿瘤发病率增加。已有报道称不同类型的肿瘤与先天性角化不良有关。我们报告第二例与霍奇金病相关的病例。该病例的其他不寻常特征包括皮肤体征出现延迟以及与慢性乙型肝炎相关。
