关键词: 弹性组织, 皮肤表现, 丘疹性弹性纤维离解

Abstract: 【Abstract】 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 the 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.

Key words: Elastic tissue, Skin manifestations, Papular elastorrhexis