【摘要】 报告1例局灶性真皮发育不全伴真皮上层弹性纤维溶解症。患儿男,11个月。因全身出现红色丘疹和萎缩斑11个月就诊。皮损沿Blaschko线分布,主要由毛细血管扩张、色素减退斑、萎缩斑和乳头状丘疹组成。此外,患儿还有右手中指和无名指并指畸形。皮损组织病理检查表现为表皮萎缩变薄,真皮上层水肿,毛细血管扩张。弹性纤维染色显示真皮上层的弹性纤维明显减少,而真皮中、下层的弹性纤维却基本正常。更多还原

【Abstract】 A case of focal dermal hypoplasia with upper dermal elastolysis is reported.An 11-month-old baby presented with red papules and atrophy macules all over the body.The lesions followed Blaschko’s lines and consisted of telangiectases, hypopigmentation,dermal atrophy,and tiny papillomatous papules.She also had syndactylies of the third and fourth fingers of the right hand since birth.Biopsy specimens showed epidermis was flat and atrophic,marked edema in the upper dermis. Capillary dilation and a mild perivascular lymphocytic infiltration were noticed.Verhoeff and acid orcein-Giemsa stains showed a remarkable diminution of fine elastic fibers from the papillary to upper reticular dermis,whereas those of the mid dermis were intact.更多还原