Department of Dermatology, Ruhr-University Bochum, Gudrunstr. 56, 44791 Bochum, Germany.
Arch Dermatol Res. 2010 Mar;302(2):85-93. doi: 10.1007/s00403-009-1004-0.
The clinical as well as histological data of 79 mid-dermal elastolysis (MDE) patients reported in the literature were evaluated. MDE is an acquired skin condition of the elastic tissue predominantly manifesting on the trunk and proximal extremities of young women. Most commonly observed skin changes include patches of well-circumscribed fine wrinkles (type I) and perifollicular papular protrusions (type II). Rarely, MDE may also occur with persistent reticular erythema and wrinkling (type III). The critical diagnostic histopathological feature of MDE is the selective loss of elastic fibres in the mid-dermis. Mild lymphohistiocytic infiltrates, elastophagocytosis of elastic fibres by macrophages, and even multinucleate giant cells are occasionally observed in MDE lesions. Immunohistological studies and cell culture experiments indicate that dysbalances in elastin turnover are associated with pathological degradative processes including increased elastolytic activity that finally lead to loss of elastic fibres in the mid-dermis. First-line differential diagnoses may include closely related conditions such as anetoderma, annular elastolytic giant cell granuloma, cutis laxa acquisita and pseudoxanthoma elasticum-like papillary dermal elastolysis. Future therapeutic approaches in MDE patients should focus on agents that are able to block increased elastase activity and induce elastin synthesis.
本文评估了文献中报道的 79 例中真皮弹力纤维溶解症（MDE）患者的临床和组织学数据。MDE 是一种获得性弹性组织皮肤疾病，主要表现为年轻女性的躯干和近端肢体。最常见的皮肤改变包括界限清楚的细皱纹斑（I 型）和毛囊周围丘疹性突起（II 型）。罕见情况下，MDE 也可能伴有持续的网状红斑和皱纹（III 型）。MDE 的关键诊断组织病理学特征是中真皮弹性纤维的选择性丧失。MDE 病变中偶尔可见轻度淋巴组织细胞浸润、巨噬细胞吞噬弹性纤维和多核巨细胞。免疫组织化学研究和细胞培养实验表明，弹力蛋白代谢失衡与包括弹性酶活性增加在内的病理性降解过程有关，最终导致中真皮弹性纤维丧失。一线鉴别诊断可能包括密切相关的疾病，如无弹性皮肤、环状弹性纤维溶解巨细胞肉芽肿、获得性皮肤松弛症和假性弹性纤维瘤样乳头真皮弹力纤维溶解症。MDE 患者的未来治疗方法应集中在能够阻断增加的弹性酶活性和诱导弹力蛋白合成的药物上。
