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[淋巴瘤样肉芽肿病:伴有免疫缺陷的周期性结节状淋巴瘤样脂膜炎]

[Lymphomatoid granulomatosis: cyclic nodular lymphomatoid panniculitis with immunologic deficiency].

作者信息

Hewitt J, Noble J P, Lessana-Leibowitch M, Benveniste M, Basset F, Binet O

出版信息

Ann Dermatol Venereol. 1977 Mar;104(3):231-7.

PMID:869465
Abstract

The case of a 66 years old woman having presented an unusual cyclic dermatosis of 11 months duration is reported. The cutaneous elements were successively located in a subcutaneous, dermal and epidermal situation, and presented a spontaneous healing with sometimes deep retractive residual scars after an ulceration or not. Pathologically, the lesions consisted in dense mononuclear infiltrates and vascular lesions. This disease was associated with an immuno-deficiency state characterized by extremely low levels of circulating IgM. This deficit was found to be persistant as it was still present two years later. However during this lapse of observation no cutaneous lesions recurred. The possible connexions of such a case with those of lymphomatous granulomatosis are discussed. In the absence of pulmonary lesion in the case reported here, no identification to the syndrome isolated by Liebow seems permitted. The authors offer a new denomination for this unusual entity.

摘要

报告了一例66岁女性患者,其出现了持续11个月的不寻常周期性皮肤病。皮肤病变先后出现在皮下、真皮和表皮部位,有时在溃疡后会自发愈合,留下深的收缩性残余瘢痕,有时则无溃疡。病理上,病变表现为密集的单核细胞浸润和血管病变。该疾病与免疫缺陷状态相关,其特征为循环IgM水平极低。发现这种缺陷持续存在,因为两年后仍然存在。然而,在这段观察期内,皮肤病变未复发。讨论了该病例与淋巴瘤样肉芽肿病病例的可能联系。由于此处报告的病例没有肺部病变,似乎无法确诊为利博所分离出的综合征。作者为这种不寻常的病症提供了一个新名称。

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