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[儿童深部红斑狼疮]

[Deep lupus erythematosus in children].

作者信息

Bachmeyer C, Aractingi S, Blanc F, Verola O, Dubertret L

机构信息

Clinique des maladies cutanées, Hôpital Saint-Louis, Paris.

出版信息

Ann Dermatol Venereol. 1992;119(8):535-41.

PMID:1485754
Abstract

Deep cutaneous lesions are seldom encountered in lupus erythematosus. The disease described in the literature as lupus erythematosus profundus or lupus erythematosus panniculitis usually occurs in middle-aged women. The authors report a case of deep lupus erythematosus which was exceptional in that it occurred in an 11-year old girl. The lesions were situated on the face and consisted of solid subcutaneous nodules and clear-cut ulcerations leaving atrophic pigmented scars. The histological image of a nodule was one of lobular lymphocytic panniculitis with homogeneous hyalinization of adipose nodules, to which must be added periadnexal and perivascular dermo-epidermal lymphocytic infiltrates. Granular IgM deposits arranged along the dermo-epidermal junction were observed at direct cutaneous immunofluorescence. Laboratory examinations showed leucopenia (3,300/mm3) with neutropenia (1,100/mm3) and the presence of antinuclear antibodies at 1/100 speckled fluorescence, as well as antibodies directed against native DNA. Studies of renal function and for complement gave normal results. The other causes of lobular panniculitis were excluded. The lesions regressed within 3 weeks under hydroxychloroquine; this drug was also successful in arresting a relapse consecutive to withdrawal of treatment. The authors have analysed the 17 paediatric cases of deep lupus erythematosus and were able to determine their main characteristics: 1. The lesions occur mostly in girls (70 p. 100). They are located electively on the face and the lateral aspect of the shoulders. They consist of well-limited, solid or hard subcutaneous nodule which may congregate to form plaques. The epidermis may be normal or pathological, poikilodermic, looking like a discoid or ulcerated lupus erythematosus. The lesions regress, leaving a characteristic atrophic scar.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

深部皮肤病变在红斑狼疮中很少见。文献中描述的深部红斑狼疮或狼疮性脂膜炎通常发生在中年女性。作者报告了一例深部红斑狼疮病例,该病例的特殊之处在于发生在一名11岁女孩身上。病变位于面部,由坚实的皮下结节和边界清晰的溃疡组成,留下萎缩性色素沉着瘢痕。结节的组织学图像为小叶性淋巴细胞性脂膜炎,脂肪结节有均匀的玻璃样变,此外还有附件周围和血管周围的真皮-表皮淋巴细胞浸润。直接皮肤免疫荧光检查观察到沿真皮-表皮交界处有颗粒状IgM沉积。实验室检查显示白细胞减少(3300/mm³)伴中性粒细胞减少(1100/mm³),抗核抗体呈斑点状荧光1/100阳性,以及抗天然DNA抗体阳性。肾功能和补体检查结果正常。排除了小叶性脂膜炎的其他病因。在羟氯喹治疗下,病变在3周内消退;该药物在阻止停药后复发方面也取得了成功。作者分析了17例儿童深部红斑狼疮病例,并确定了其主要特征:1. 病变大多发生在女孩(70%)。它们选择性地位于面部和肩部外侧。由界限清楚的坚实或硬皮下结节组成,这些结节可聚集形成斑块。表皮可能正常或病理改变,呈异色性皮肤,类似盘状或溃疡性红斑狼疮。病变消退后留下特征性萎缩性瘢痕。(摘要截取自250字)

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