Lopez C, Oliver M, Olavarria R, Sarabia M A, Chopite M
Department of Dermatopathology, Instituto de Biomedicina, Hospital Vargas, Universidad Central de Venezuela, Caracas.
Am J Dermatopathol. 2000 Aug;22(4):328-33. doi: 10.1097/00000372-200008000-00006.
Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease is a rare entity, and even more rarely, it is associated with other diseases. In a few cases, the condition is associated with cutaneous lupus erythematosus (CLE). Histiocytic necrotizing lymphadenitis predominantly affects young women, who present with solitary or multiple cervical lymphadenopathy accompanied by symptoms such as fever, weight loss, sweating, or, in exceptional cases, hepatosplenomegaly. Laboratory examinations show normal or nonspecific results. The disease is of unknown origin, although a viral origin has been suggested, with the suspected agents including Epstein-Barr virus, herpesvirus type 6, and cytomegalovirus (CMV). Although the first and most of the more recent cases have been reported in Oriental patients, the disease has a wide geographic distribution. The clinical evolution is favorable, with spontaneous remission in less than 4 months in almost all cases. We present a case of a 37-year-old woman from Peru who presented with cervical adenopathies on two occasions. Biopsy of a lymph node revealed a histopathologic picture compatible with Kikuchi-Fujimoto histiocytic necrotizing lymphadenitis. The adenopathy disappeared in a few months. A year later, she presented with a maculopapular rash in the nasal and malar regions. The results of the skin biopsy and immunofluorescence examination were compatible with chronic CLE. The results of the serology testing for CMV were positive. Treatment with chloroquine was initiated, with almost complete recovery by 5 months. No manifestations of systemic lupus erythematosus have occurred since. The epidemiologic, clinical, and anatomopathologic aspects as well as the differential diagnosis of this entity are reviewed.
组织细胞坏死性淋巴结炎或菊池-藤本病是一种罕见疾病，更罕见的是它与其他疾病相关。在少数情况下，该病症与皮肤红斑狼疮（CLE）有关。组织细胞坏死性淋巴结炎主要影响年轻女性，她们表现为单个或多个颈部淋巴结病，并伴有发热、体重减轻、出汗等症状，在极少数情况下还伴有肝脾肿大。实验室检查结果正常或无特异性。尽管有人提出该病起源于病毒，怀疑的病原体包括爱泼斯坦-巴尔病毒、6型疱疹病毒和巨细胞病毒（CMV），但其病因不明。尽管首例及大多数近期病例报告来自东方患者，但该病在地理上分布广泛。临床病程良好，几乎所有病例在不到4个月内自发缓解。我们报告一例来自秘鲁的37岁女性，她曾两次出现颈部淋巴结病。淋巴结活检显示组织病理学图像与菊池-藤本组织细胞坏死性淋巴结炎相符。淋巴结病在几个月内消失。一年后，她在鼻和颊部出现斑丘疹皮疹。皮肤活检和免疫荧光检查结果与慢性CLE相符。CMV血清学检测结果呈阳性。开始用氯喹治疗，5个月时几乎完全康复。此后未出现系统性红斑狼疮的表现。本文对该疾病的流行病学、临床和解剖病理学方面以及鉴别诊断进行了综述。
