单侧性真皮中层弹性组织溶解症一例

A case of uafilateral mid-dermal elastolysis

患者女，38岁，颈右侧黄色丘疹伴带状萎缩20年。皮损缓慢发展，无明显症状。否认有长期日晒及家族史。皮肤科检查：颈右前侧皮肤见沿皮纹方向、大小不一的黄色丘疹、斑块；颈右后侧见多数以毛囊为中心的针帽大丘疹；整个颈右侧皮肤呈境界较清楚的带状萎缩斑，表面为细皱纹样改变，似老年人皮肤外观。颈部左侧皮肤大致正常，黄色丘疹处组织病理检查：表皮大致正常；真皮浅层血管周稀疏淋巴组织细胞及少量噬黑素细胞浸润，未见日光性变性改变；真皮中层胶原纤维增粗，排列紧密，未见明显炎细胞浸润。弹力纤维染色示真皮网状层弹性组织几乎完全缺失，剩余的少量弹性纤维肿胀、断裂明显。VonKossa染色阴性。诊断：单侧性真皮中层弹性组织溶解症。

A 38-year-old woman presented with a 20-year history of yellow papules and band-like atrophy on the right neck. The lesions developed slowly and were asymptomatic. There was no history of long-term sun exposure or family history of similar diseases. Skin examination revealed multiple irregularly sized yellow papules and plaques on the right anterior neck following cleavage lines, multiple pin cap-sized perifollicular papules on the posterior right neck. Well-defined band-like atrophic patches with fine wrinkling were observed in the whole right neck, giving an aged appearance. The skin of the left neck was nearly normal. Pathological examination of biopsy specimens from the yellow papules showed a normal epidermis, scant lymphohistiocytic and melanophage infiltrates around the vessels in the superficial dermis without solar degradation. The collagen bundles in the mid dermis were slightly thickened and arranged tightly in parallel to the skin surface with the absence of inflammatory infiltrate. Verhoeff-van Giesen＇s staining confirmed a nearly complete absence of elastic fibers in the mid dermis as well as obvious swelling and breakage of resident scant elastic fibers. Von Kossa＇s staining was negative. Based on the above findings, the diagnosis was made as unilateral mid-dermal elastolysis.