Le Scanff Julie, Sève Pascal, Dalle Stephane, Kodjikian Laurent, Thomas Luc, Broussolle Christiane
Department of Internal Medicine, Hotel Dieu, Lyon, France.
Int J Dermatol. 2007 Jun;46(6):622-4. doi: 10.1111/j.1365-4632.2006.03004.x.
Pseudoxanthoma elasticum (PXE) is a heritable connective tissue disorder affecting the elastic structures in the body, manifesting with cutaneous, ophthalmologic and cardiovascular findings. The association of PXE and systemic disease is rare, as only six cases have been reported in the literature. We describe herein two cases of coexisting PXE and cutaneous lupus and discuss a possible association. Although a fortuitous association cannot be excluded, the calculated prevalence of the association in our area was much less than in our observation. Given the ocular and vascular complications of PXE, clinicians who treat patients with lupus should be aware of this possible association.
弹性假黄瘤(PXE)是一种遗传性结缔组织疾病,会影响身体的弹性结构,表现为皮肤、眼科和心血管方面的症状。PXE与全身性疾病的关联较为罕见,文献中仅报道过6例。我们在此描述2例同时存在PXE和皮肤狼疮的病例,并探讨一种可能的关联。尽管不能排除偶然关联的可能性,但在我们所在地区计算出的这种关联的患病率远低于我们的观察结果。鉴于PXE的眼部和血管并发症,治疗狼疮患者的临床医生应意识到这种可能的关联。