Taylor Nancy E, Foster William C, Wick Mark R, Patterson James W
Department of Pathology, University of Virginia Health System, Charlottesville, VA, USA.
J Cutan Pathol. 2004 Feb;31(2):205-9. doi: 10.1111/j.0303-6987.2004.00168.x.
Lipedema is a condition characterized by diffuse, bilaterally symmetrical, painful swelling of the legs and buttocks. Microscopically, there are dermal and septal edema, adipocyte degeneration, and numerous mast cells, features held in common with lipedematous alopecia.
We present the case of a 60-year-old woman with a long history of bilateral leg masses with microscopic features of lipedema. In addition, elastic-fiber changes typical of pseudoxanthoma elasticum (PXE) were discovered within the subcutaneous septa in three separate specimens obtained from an affected extremity. The patient did not have other clinical findings of PXE, although there was a history of both hypertension and congestive heart failure.
This tumefactive presentation of lipedema has not been previously described. Regarding the elastic-tissue abnormalities, the patient could have either a subclinical form of PXE, perhaps predisposing to lipedema, or secondary elastic-tissue changes resulting from the massive edema. If the latter is the case, then this could represent an unusual manifestation of localized acquired cutaneous PXE (calcific elastosis).
脂肪性水肿是一种以双下肢及臀部弥漫性、双侧对称性、疼痛性肿胀为特征的病症。显微镜下可见真皮及间隔水肿、脂肪细胞变性以及大量肥大细胞,这些特征与脂肪性水肿性脱发相同。
我们报告一例60岁女性患者,其双侧腿部肿物病史较长,具有脂肪性水肿的微观特征。此外,在取自患侧肢体的三个独立标本的皮下间隔内发现了弹性假黄瘤(PXE)典型的弹性纤维改变。尽管患者有高血压和充血性心力衰竭病史,但并无PXE的其他临床症状。
这种脂肪性水肿的肿胀表现此前未见报道。关于弹性组织异常,该患者可能患有PXE的亚临床形式,或许这是导致脂肪性水肿的原因,或者是由大量水肿引起的继发性弹性组织改变。如果是后者,那么这可能代表局限性后天性皮肤PXE(钙化弹性组织变性)的一种不寻常表现。