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硬皮病样线状狼疮性脂膜炎:两例报告

Sclerodermic linear lupus panniculitis: report of two cases.

作者信息

Marzano A V, Tanzi C, Caputo R, Alessi E

机构信息

Institute of Dermatological Sciences of the University of Milan and IRCCS Ospedale Maggiore of Milan, Milan, Italy.

出版信息

Dermatology. 2005;210(4):329-32. doi: 10.1159/000084760.

Abstract

Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or 'overlap' forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus panniculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, 'sclerodermic' subset of lupus erythematosus panniculitis.

摘要

红斑狼疮性脂膜炎是一种罕见疾病,其特征为深部皮下结节,最常见于上肢和面部。已有报道称其具有独特的临床表现,如线性形态或红斑狼疮性脂膜炎与局限性硬皮病之间的“重叠”形式。我们在此呈现2例具有线性红斑狼疮性脂膜炎并伴有局限性硬皮病样改变患者的临床特征、病程及实验室检查结果。这2例患者(自1990年以来我们所诊治的14例红斑狼疮性脂膜炎患者中的2例)均为女性,发病年龄较轻(中位年龄25岁)。其中1例病情进展为伴有严重肾脏受累的系统性红斑狼疮,而另1例患者发生自然流产且出现抗心磷脂抗体,应密切随访并筛查抗磷脂综合征的发生。因此,与通常被认为是良性疾病的红斑狼疮性脂膜炎的一般病程相比,这种变异型的临床行为似乎更具侵袭性,尽管一些报告表明其预后并非总是良好。线性分布可能是红斑狼疮性脂膜炎这种独特的“硬皮病样”亚型的临床标志。

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