摘要
目的探讨反应性穿通性胶原病(reactive penetrating collagenosis, RPC)的临床表现、伴随疾病以及治疗反应。方法回顾性分析西安交通大学第二附属医院2014年11月-2020年8月经组织病理确诊的28例RPC患者的病史、皮损特点、伴随疾病、家族史及治疗反应等资料。结果 28例患者的平均发病年龄为44岁,病程中位数为1年,发病无性别差异,且均无家族史。皮损最常见于下肢(85.71%),常为多发(89.29%),但也可单发于阴囊、腰背部及足部等。表现为瘙痒或疼痛性红斑、丘疹、溃疡,边缘堤状隆起,中央凹陷上覆不易剥离的角栓,可伴有同形反应(14.29%)。12例(42.86%)患者合并一种或多种其他系统疾病,以糖尿病、高血压、慢性肾衰竭为主。组织病理可见变性胶原纤维从表皮垂直穿出。治疗主要为控制原发病、抗炎、抗感染、对症、抑制异常角化等。除3例患者手术切除后缓解及1例失访者外,其余24例药物治疗者中仅12例(50.00%)治愈,随访无复发。结论 RPC临床较少见,皮损常多发于双下肢,但也可单发于特殊部位或局限于单侧肢体。儿童发病可为特发性或遗传性,成人发病多为获得性,也可无伴随疾病,其发病机制不清,尚无特效治疗方法。
Objective To investigate the clinical manifestations,concomitant diseases and therapeutic reaction of reactive penetrating collagenosis(RPC).Methods The medical history,skin lesion characteristics,concomitant diseases,family history and therapeutic reaction of 28 RPC patients diagnosed by histopathology in the Second Affiliated Hospital of Xi'an Jiaotong University from November 2014 to August 2020 were retrospectively analyzed.Results The mean age of onset of the 28 patients was 44 years old,and the median course of disease was one year.There was no gender difference in terms of onset of disease,and none of them had a family history.Skin lesions were most common on lower extremities(85.71%),and mostly multiple(89.29%),but single lesions could also be found on scrotum,lower back and feet.Clinical manifestations were pruritus or painful erythema,papule,ulcer,peripheral bulges and central depression covered with hard-to-remove keratinization,which might be accompanied by Koebner's phenomenon(14.29%).Twelve patients(42.86%)had one or more other systemic diseases,mainly diabetes mellitus,hypertension and chronic renal failure.Histopathology showed the denatured dermal collagen penetrated vertically from the epidermis.The treatment mainly included the treatment of primary disease,anti-inflammatory,anti-infection and symptomatic treatment,and inhibition of abnormal keratosis.In addition to three patients with remission after surgical resection and one patient who was lost to follow-up,only 12(50.00%)of the remaining 24 patients were cured and no recurrence was found during follow-up.Conclusion RPC is a rare disease.The skin lesions usually occur on both lower extremities,but also can be single lesions on specific areas or confined to one limb.The disease can be idiopathic or hereditary in children,and mostly acquired in adults,with or without accompanying diseases.The pathogenesis is unclear,and there is no specific treatment.
作者
拓惠惠
郑焱
张婧
王宁
杨鹏举
杨雅杰
杜文倩
孙妍
冯义国
TUO Huihui;ZHENG Yan;ZHANG Jing;WANG Ning;YANG Pengju;YANG Yajie;DU Wenqian;SUN Yan;FENG Yiguo(Department of Dermatology,the Second Affiliated Hospital of Xi'an Jiaotong University,Xi'an 710004,China)
出处
《中国皮肤性病学杂志》
CSCD
北大核心
2021年第9期1001-1006,共6页
The Chinese Journal of Dermatovenereology
关键词
反应性穿通性胶原病
临床特点
伴随疾病
治疗方法
Reactive penetrating collagenosis
Clinical characteristics
Concomitant disease
Treatment
