苯妥英钠联合沙利度胺治疗胫前型营养不良型大疱性表皮松解症1例  

Efficacy of Phenytoin Combined with Thalidomide in the Treatment of Pretibial Dystrophic Epidermolysis Bullosa:A Case Report

作　　者：赵巍[1] 李瑞琴[2] 孟威威 王丽[1] 王庆兴[1] 方玉甫[1] ZHAO Wei;LI Ruiqin;MENG Weiwei;WANG Li;WANG Qingxing;FANG Yufu(Department of Dermatology,the Second Affiliated Hospital of Henan University of Chinese Medicine,Henan Provincial Hospital of Traditional Chinese Medicine,Zhengzhou 450000,China;Pathology Clinical Teaching and Research Base of Henan University of Traditional Chinese Medicine,Zhengzhou 450000,China)

机构地区：[1]河南中医药大学第二附属医院河南省中医院皮肤科,河南郑州450000 [2]河南中医药大学病理学临床教学科研基地,河南郑州450000

出　　处：《中国皮肤性病学杂志》2022年第3期307-310,共4页The Chinese Journal of Dermatovenereology

基　　金：河南省中医专科诊疗中心建设项目(豫中医医政[2020]63号)。

摘　　要：患者女,46岁,双下肢丘疹、水疱、结节20余年。趾甲逐渐出现变黄、变形、增厚。组织病理检查示:表皮下水疱,周围散在淋巴细胞,未见嗜酸性粒细胞。表皮无细胞水肿,无棘层松解,真皮及血管周围无明显炎症细胞浸润。直接免疫荧光:C3、C4、IgG、IgA(-)。最终诊断:胫前型营养不良型大疱性表皮松解症。患者经糖皮质激素、免疫抑制剂、中药内服多次治疗后皮疹仍有反复,伴明显瘙痒。随后,尝试使用苯妥英钠及沙利度胺等治疗19周后,皮损基本消退,无明显瘙痒,现仍在随访中。A 46-year-old female prsented with pimples,blisters and nodules in both lower limbs for more than 20 years.The toenails gradually turned yellow,deformed and thickened.Histopathological examination showed subcutaneous blisters with scattered lymphocytes and no eosinophils.There was no cell edema and acantholysis in epidermis,and no obvious inflammatory cell infiltration around dermis and blood vessels.Direct immunofluorescence showed the C3,C4,IgG,and IgA(-).The final diagnosis was pretibial dystrophic epidermolysis bullosa.After repeated treatment with glucocorticoid,immunosuppressant and traditional Chinese medicine,the rash was still recurrent,accompanied by obvious itching.The phenytoin and thalidomide treatment were tried for 19 weeks,the rash were subsided,and the pruritus was significantly relieved.The patient was still under the follow-up.

关 键 词：营养不良型大疱性表皮松解症 胫前型 苯妥英钠 沙利度胺 

分 类 号：R758.59[医药卫生—皮肤病学与性病学]