着色性干皮病(xeroderma pigmen-tosum,简称XP)是一种罕见的遗传性疾病,其主要临床特征为皮肤广泛的色素性皮疹、萎缩及角化,尤以裸露部位为重,易发生癌变。据美国、日本等国报道,本病患者皮肤癌的发病率几乎是100％。作者在本区发现二个XP家系4例患者,现报告如下: 病例报告及家系分析家系1:先证者(图1Ⅳ_1)男,7岁,回族, Xeroderma pigmen-tosum (referred to as XP) is a rare genetic disease, the main clinical features of the skin is a wide range of pigmented rash, atrophy and keratosis, especially in the bare parts of the weight, prone to cancer . According to the United States, Japan and other countries reported that the incidence of skin cancer in patients with this disease is almost 100%. The authors found two XP families in this area, four patients are reported as follows: Case reports and pedigree Analysis of family 1: proband (Figure 1Ⅳ_1) male, 7 years old, Hui,