Chua S H, Giam Y C, Sim C S
National Skin Centre, Singapore.
Ann Acad Med Singap. 1996 Jul;25(4):599-601.
A 9-year-old Chinese girl with systemic lupus erythematosus presented initially with persistent cervical lymphadenopathy without any constitutional symptoms. The histology of the involved lymph node showed histiocytic necrotizing lymphadenitis and the initial clinical diagnosis of Kikuchi-Fujimoto disease was made. The patient subsequently developed fever, oral ulcerations, epistaxis, generalised myalgia and erythema multiforme-like skin lesions over the face and upper arms. Investigations showed anaemia, leucopenia, hypocomplementemia, a positive antinuclear antibody and a positive anti-double-stranded DNA antibody test. The clinical diagnosis was revised to systemic lupus erythematosus. The histological picture of histiocytic necrotizing lymphadenitis can occur in systemic lupus erythematosus and may be indistinguishable from that seen in Kikuchi-Fujimoto disease. The diagnosis of Kikuchi-Fujimoto disease should be confined to a benign clinical subset of histiocytic necrotizing lymphadenitis which resolves spontaneously without treatment. The occurrence of erythema multiforme-like lesions in lupus erythematosus is unusual and together with the characteristic serologic abnormalities, fits Rowell's syndrome.
一名9岁的中国系统性红斑狼疮女孩最初表现为持续性颈部淋巴结肿大，无任何全身症状。受累淋巴结的组织学检查显示为组织细胞坏死性淋巴结炎，初步临床诊断为菊池-藤本病。该患者随后出现发热、口腔溃疡、鼻出血、全身肌痛以及面部和上臂出现多形红斑样皮肤损害。检查显示贫血、白细胞减少、补体血症降低、抗核抗体阳性以及抗双链DNA抗体检测阳性。临床诊断修订为系统性红斑狼疮。组织细胞坏死性淋巴结炎的组织学表现可出现在系统性红斑狼疮中，可能与菊池-藤本病所见难以区分。菊池-藤本病的诊断应局限于组织细胞坏死性淋巴结炎的一个良性临床亚组，该亚组无需治疗即可自发缓解。系统性红斑狼疮中出现多形红斑样损害并不常见，结合特征性的血清学异常，符合罗威尔综合征。
