González-López Marcos A, Drake Marta, González-Vela M Carmen, Armesto Susana, Llaca Héctor F, Val-Bernal J Fernando
Dermatology Service, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Avda. de Valdecilla s/n, E-39008 Santander, Spain.
J Dermatol. 2006 Oct;33(10):709-13. doi: 10.1111/j.1346-8138.2006.00165.x.
We present further evidence that generalized morphea (GM) and primary biliary cirrhosis (PBC) may be associated. As far as we know, only six cases with this association have been previously reported in the published work, all of which were observed in women. We describe the case of a 62-year-old man diagnosed with M2-antibody-positive PBC who developed multiple generalized indurated plaques on the trunk and extremities 3 years later. Clinical history, laboratory data and histopathological examination were consistent with the diagnosis of GM. The coexistence in a male of these two entities that predominantly affect females reinforces the hypothesis that a pathogenic link exists between GM and PBC. Consequently, PBC should be looked for in all patients with GM.
我们提供了进一步的证据表明，泛发性硬斑病（GM）和原发性胆汁性肝硬化（PBC）可能存在关联。据我们所知，此前发表的文献中仅报道过6例这种关联病例，所有病例均见于女性。我们描述了一例62岁男性患者，其被诊断为M2抗体阳性的PBC，3年后在躯干和四肢出现了多个泛发性硬结性斑块。临床病史、实验室数据和组织病理学检查均符合GM的诊断。这两种主要影响女性的疾病在男性患者中共存，强化了GM与PBC之间存在致病联系的假说。因此，对于所有GM患者都应筛查是否存在PBC。
