摘要
患者女,56岁,全身皮肤色素异常伴瘙痒4年。皮肤科情况:面部皮肤肿胀并多发扩张性毛细血管;躯干、四肢皮肤肿胀、发硬并弥漫性色素沉着斑夹杂色素减退斑,呈"盐和胡椒"征象。皮损组织病理示:表皮角化过度,棘层局限性变薄;真皮内胶原红染、致密、玻璃样变性,血管及附属器周围可见挤压现象及淋巴细胞浸润。肝脏组织病理示:汇管区纤维组织增生,慢性炎细胞浸润,轻-中度界板炎;肉芽肿形成,局灶小胆管损伤。诊断:系统性硬化症合并原发性胆汁性胆管炎重叠综合征。系统使用糖皮质激素、免疫抑制剂和熊去氧胆酸联合治疗3周后症状明显缓解。
A 56-year-old female patient presented with skin pigmentation and pruritus for four years.Skin examination revealed the swelling and multiple dilated capillaries in the skin of face.Swelling,hardening and diffuse pigmentation and hypopigmentation were found in the skin of trunk and limbs,showing the sign of"salt and pepper".Histopathological examination of the skin lesion showed the epidermis hyperkeratosis and local thinning of spinous layer.And the collagen in the dermis was red-stained,dense and hyaline degeneration,and there were extrusion phenomenon and lymphocyte infiltration around blood vessels and appendages.Liver histopathologic examination showed the hyperplasia of fibrous tissue and chronic inflammatory cell infiltration in the portal area,presenting a mild to moderate inflammation.At the same time,granuloma formation and focal small bile duct injury were observed.Diagnosis:systemic sclerosis-primary biliary cholangitis overlap syndrome.After three weeks of systemic treatment with glucocorticoids,immunosuppressants and ursodeoxycholic acid,the symptoms were significantly relieved.
作者
卞璐
张莎莎
王建波
洪帆
张友春
李敏
张守民
BIAN Lu;ZHANG Shasha;WANG Jianbo;HONG Fan;ZHANG Youchun;LI Min;ZHANG Shoumin(Department of Dermatology,Henan Provincial People's Hospital,Zhengzhou University People's Hospital,Zhengzhou 450003,China;Healthcare Cadre Institute of Henan,Zhengzhou 450003,China;Department of Pathology,Henan Provincial People's Hospital,Zhengzhou 450003,China)
出处
《中国皮肤性病学杂志》
CAS
CSCD
北大核心
2021年第6期657-660,共4页
The Chinese Journal of Dermatovenereology
基金
河南省医学科技攻关计划省部共建青年项目(SB201904011)。
