丘疹性弹性纤维离解22例临床病理分析

Clinicopathological analysis of 22 cases of papular elastorrhexis

目的分析丘疹性弹性纤维离解的临床及病理学特点。方法回顾2006年9月至2018年5月在中国医学科学院皮肤病医院确诊的22例丘疹性弹性纤维离解患者的临床、病理学表现及随访情况。结果22例患者平均发病年龄5.7岁(1~10岁),男女比例4.5∶1,发病至确诊时间平均1.5年,无明确病因。患者皮损均无痒痛等症状,均表现为白色丘疹,直径1~10 mm,圆形、椭圆形或多角形,轻度隆起,质软,境界清晰,推挤丘疹两侧正常皮肤后丘疹表面可出现皱纹。16例(73%)皮损散在分布,13例(59%)皮损数<5个;21例(95%)皮损位于躯干部位。8例皮损组织病理表现:真皮浅中层胶原纤维无明显增加,排列正常,但间隙轻度增宽;弹性纤维染色显示真皮浅中层局限性弹性纤维消失及离解。确诊后,22例均未使用任何药物治疗,其中18例自起病后皮损未继续扩大,也未出现新发皮损;4例皮损轻度扩大,但后期停止进展;16例患者出现部分缓解。结论丘疹性弹性纤维离解是一种好发于儿童青少年的罕见弹性纤维性皮肤病,诊断需临床结合病理,无需特殊治疗,预后良好。

Objective To analyze clinical and pathological features of papular elastorrhexis.Methods Clinical data were collected from 22 patients with confirmed papular elastorrhexis in Hospital for Skin Diseases,Chinese Academy of Medical Sciences and Pekin Union Medical College from September 2006 to May 2018.Clinical manifestations,pathological findings and follow-up results were retrospectively analyzed.Results The average age of onset of the 22 patients was 5.7 years(range:1-10 years),and the male to female ratio was 4.5∶1.The average duration from the occurrence of disease to the confirmation of diagnosis was 1.5 years,and no definite etiology was found.The patients had no itching or pain sensation.Skin lesions were soft,slightly elevated,well-circumscribed,round,oval or polygonal-shaped,white papules with diameters of 1-10 mm,and wrinkles appeared on the surface of the papule when the papule was pushed towards its center.Among the 22 patients,16(73%)presented with scattered lesions,13(59%)had less than 5 papules,and lesions were located in the trunk in 21(95%).Histopathological examination of skin lesions in 8 patients showed no obvious increase of collagen fibers in the superficial and middle dermis,which were normally arranged with slightly widened spaces between them.Elastic fiber staining showed that elastic fibers disappeared or were dissociated focally in the superficial and middle dermis.After confirmed diagnosis,the 22 patients received no treatment.In 18 patients,skin lesions did not continue to expand after onset,and no new skin lesions occurred.Skin lesions were slightly enlarged,but remained steady thereafter in 4 patients.Sixteen patients achieved partial remission.Conclusions Papular elastorrhexis is a rare skin disorder of elastic fibers that occurs predominantly during childhood and adolescence,and its diagnosis relies on clinical manifestations combined with histopathological findings.No special treatment is needed and the prognosis is good.