单侧多发线状汗孔角化症合并布-加氏综合征1例

A Case of Linear Porokeratosis Associated with Budd-Chiari Syndrome

患者女,41岁。右侧躯干、上肢皮肤起疹20年,肝脾肿大6年,呕血、黑便20天。体检:慢性肝病面容,颈静脉怒张,腹部膨隆,可见胸壁及腹壁静脉曲张。皮肤科情况:右侧胸部、右背部、右上肢皮肤可见数条带状分布的褐色呈环状斑丘疹,边缘呈堤状隆起,部分皮疹表面破溃、结痂。皮损组织病理示:表皮内可见角化不全柱,其下可见坏死角质形成细胞,真皮浅层血管周围可见黑色素沉积。肝胆胰CT平扫和三期增强及腔静脉造影检查证实为布-加氏综合征。诊断:单侧多发线状汗孔角化症合并布-加氏综合征。

A 41-year-old female presented with rash on the right trunk and upper limb for 20 years,liver and spleen enlargement for 6 years,and haematemesis and melena for 20 days. Physical examination showed chronic hepatic facies,distention of jugular venous and abdominal,varicose veins of the chest wall and abdominal wall. Dermatological examination showed several bands of brown maculopapule on the right side of the chest,right back and right upper limb. The edge embanked,and with ulcerationand crusts. Skin pathology revealed cornoid lamellae in epidermis,under which the necrotic keratinocytes can be seen. The melanin deposits around the superficial vessels of dermis. CT and Vena cava angiography proved Budd-Chiars syndrome. The diagnose of Linear porokeratosis associated with Budd-Chiars syndrome was made.