色素血管性斑痣性错构瘤病一家系报告  

Phakomatosis Pigmentovascularis Reported in One Family

作　　者：赵文琪 卜晓琳[2] 邬思远 ZHAO Wenqi;BU Xiaolin;WU Siyuan(Ningxia Medical University,Postgraduate Training Base in Shanghai Gongli Hospital,Shanghai 200135,China;Department of Dermatology,Shanghai Pudong New Area Gongli Hospital,Shanghai 200135,China)

机构地区：[1]宁夏医科大学,上海市浦东新区公利医院研究生培养基地,上海200135 [2]上海市浦东新区公利医院皮肤性病科,上海200135

出　　处：《中国皮肤性病学杂志》2022年第9期1079-1082,共4页The Chinese Journal of Dermatovenereology

基　　金：国家自然科学基金(81703151);浦东新区卫生系统学科带头人培养计划(PWRd2018-14)。

摘　　要：先证者男,43岁,出生时躯干片状青斑,随年龄增长皮疹不退,逐渐覆盖几乎全部躯干和四肢,13岁时上肢出现红斑。皮疹现表现为躯干、双下肢大片青灰色斑片,颈部、右上臂、腋下网状分布红斑,部分与青灰斑重叠,压之褪色。其子7岁,躯干青灰斑的发展过程及表现与先证者类似,目前未有红斑出现。The proband was a 43-year-old male,who was born with flaky cyan spots on the trunk,and the rash does not recede with age,gradually covering almost all of the trunk and limbs.Erythema appeared on the upper limbs at the age of 13.The rash presented as a large bluish-gray patch on the trunk and lower limbs.Erythema was distributed in a reticulum of the neck,right upper arm,and armpit,and some overlap with the bluish-gray spots,and faded under pressure.The son of the proband was 7 years old,and the development process and performance of the bluish-gray spots on the trunk were similar to those of the proband,and no erythema has been seen at present.

关 键 词：色素血管性斑痣性错构瘤病 血管畸形 并发症 

分 类 号：R739.5[医药卫生—肿瘤]