Andrés-Ramos Irene, Alegría-Landa Victoria, Gimeno Ignacio, Pérez-Plaza Alejandra, Rütten Arno, Kutzner Heinz, Requena Luis
Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.
Dermatopathology Laboratorie, Friedrichschafen, Germany.
Am J Dermatopathol. 2019 Feb;41(2):85-117. doi: 10.1097/DAD.0000000000001275.
After a review of the physiology in the formation and degradation of cutaneous elastic tissue, we describe the clinicopathologic disorders characterized by increased and decreased cutaneous elastic tissue. Cutaneous disorders characterized by increased and/or abnormal elastic tissue in the dermis include elastoma, also named nevus elasticus, dermatosis lenticularis disseminata, pseudoxanthoma elasticum, late-onset focal dermal elastosis, linear focal elastosis, elastoderma, elastofibroma dorsi, and elastosis perforans serpiginosa. In some of these conditions, the specific histopathologic diagnosis may be rendered with hematoxylin-eosin stain, whereas in other ones special elastic tissue stains are necessary to demonstrate the anomalies. Cutaneous disorders characterized by decreased dermal elastic tissue include nevus anelasticus, papular elastorrhexis, perifollicular elastolysis, anetoderma cutis laxa, postinflammatory elastolysis and cutis laxa, white fibrous papulosis of the neck, pseudoxanthoma elasticum-like papillary dermal elastolysis, and mid dermal elastolysis. In most of these conditions, the histopathologic anomalies are only seen with elastic tissue stains, and cutaneous biopsies of these processes stained with hematoxylin-eosin show appearance of normal skin. The diagnosis of some of these disorders characterized by increased or decreased elastic dermal tissue should be followed by general exploration of the patient to rule out associated severe systemic anomalies, and in some cases, a genetic counseling should be offered to the family.
在回顾了皮肤弹性组织形成和降解的生理学过程后,我们描述了以皮肤弹性组织增加和减少为特征的临床病理紊乱。以真皮中弹性组织增加和/或异常为特征的皮肤疾病包括弹性瘤(也称为弹性痣)、播散性豆状皮肤纤维瘤病、弹性假黄瘤、迟发性局灶性真皮弹力组织变性、线状局灶性弹力组织变性、弹力性皮肤病、背部弹力纤维瘤和匐行性穿通性弹力纤维病。在其中一些情况下,苏木精-伊红染色即可作出特异性组织病理学诊断,而在其他情况下,则需要特殊的弹性组织染色来显示异常。以真皮弹性组织减少为特征的皮肤疾病包括无弹性痣、丘疹性弹力纤维松解症、毛囊周围弹力纤维溶解症、皮肤松弛症、炎症后弹力纤维溶解症和皮肤松弛症、颈部白色纤维性丘疹病、弹性假黄瘤样乳头真皮弹力纤维溶解症和真皮中部弹力纤维溶解症。在大多数这些情况下,组织病理学异常仅在弹性组织染色时可见,用苏木精-伊红染色的这些病变的皮肤活检显示为正常皮肤外观。对于一些以真皮弹性组织增加或减少为特征的疾病,诊断后应全面检查患者以排除相关的严重全身异常,在某些情况下,应向患者家属提供遗传咨询。