Schnitzler L, Abimelech P H, Naveau B
Ann Dermatol Venereol. 1988;115(6-7):679-84.
The finding in a 40-year old and apparently healthy woman of a pseudotumoral lesion of the left arm which had gradually developed over a 9-year period led to a tentative clinical diagnosis of lupus anetoderma. This diagnosis was confirmed on the one hand by histological examination of the skin--which showed almost total disappearance of dermal elastic fibres, while the lympho-histiocytic infiltrate was so discreet that it excluded a deep lupus--and on the other hand by direct immunofluorescence, with lupus band on the lesion and a few abnormalities of the exposed and covered healthy skin. A history of chorea at the age of 13 years raised the problem of a possible relationship between this neurological disease and a systemic lupus erythematosus. This hypothesis was supported by resolutive episodes of arthralgia and abnormal laboratory findings, such as accelerated ESR, leucopenia, decrease of complement and presence of homogeneous antinuclear antibodies. Anticardiolipin antibodies, circulating anticoagulants and VDRL test were negative. Treatment with anti-malarials brought about some degree of cutaneous shrinkage and a significant decrease in ESR. This case is reported because anetoderma as sole manifestation of systemic lupus erythematosus and the occurrence, 17 years previously, of chorea are exceptional events.
一名40岁看似健康的女性,其左臂出现一个假性肿瘤性病变,该病变在9年时间里逐渐发展,这一发现使得临床初步诊断为皮肤松弛性狼疮。一方面,皮肤组织学检查证实了这一诊断——显示真皮弹性纤维几乎完全消失,而淋巴细胞-组织细胞浸润非常不明显,可排除深部狼疮;另一方面,直接免疫荧光检查显示病变部位有狼疮带,且暴露和未暴露的健康皮肤有一些异常。该患者13岁时有舞蹈病病史,这引发了这种神经系统疾病与系统性红斑狼疮之间可能存在关联的问题。关节痛的缓解期以及异常的实验室检查结果,如血沉加快、白细胞减少、补体降低和均质型抗核抗体的存在,支持了这一假说。抗心磷脂抗体、循环抗凝剂和VDRL试验均为阴性。抗疟药治疗使皮肤有一定程度的收缩,血沉显著降低。报告该病例是因为皮肤松弛作为系统性红斑狼疮的唯一表现以及17年前出现舞蹈病均为罕见情况。