迟发性皮肤卟啉病一例  

Porphyria cutanea tarda:a case report

作　　者：吉才亨 王嘉佳 王名法 罗杨[1] 刘琨 刘军麟[1] JI Cai-heng;WANG Jia-jia;WANG Ming-fa(Department of Dermatovenereology,the Second Affiliated Hospital of Hainan Medical University,Haikou 570311,China)

机构地区：[1]海南医学院第二附属医院皮肤性病科,海口570311 [2]海南医学院第二附属医院病理科,海口570311 [3]海南省人民医院皮肤性病科

出　　处：《实用皮肤病学杂志》2023年第4期248-250,共3页Journal of Practical Dermatology

摘　　要：47岁男性患者,面颈部、背部、双上肢红斑、丘疹、水疱伴瘙痒5个月余,日晒及饮酒后加重。肝功能显示转氨酶升高,尿Wood灯下呈珊瑚红色荧光。皮损组织病理:表皮下水疱形成,疱内无炎性细胞浸润,真皮下层胶原纤维增生,间有黏蛋白沉积。免疫荧光未见荧光物质沉积。全基因组外显子测序未见相关基因突变。诊断:迟发性皮肤卟啉病。给予羟氯喹、复方甘草酸苷治疗后病情缓解,随访6个月未见复发。A 47-year-old male patient presented with erythema,papules,and blisters on his face,neck,back,and both upper extremities,accompanied by itchiness for more than 5 months.The lesions were aggravated by sun exposure and alcohol consumption.Liver function tests showed elevated transaminases,and the urine exhibited coral-red fluorescence under Wood's lamp.Whole-genome exome sequencing revealed no gene mutations.Histopathology of skin lesions revealed subepidermal blisters(without inflammatory cell infiltration),mucin deposition,and proliferation of collagen fibers in the subdermal layer.No fluorescent material deposition was observed by immunofluorescence.A final diagnosis of porphyria cutanea tarda was made.The symptoms were relieved after treatment with hydroxychloroquine and compound glycyrrhizin.No recurrence was observed during the 6-month follow-up.

关 键 词：皮肤卟啉病 迟发性 

分 类 号：R589.8[医药卫生—内分泌]