Masab Muhammad, Surmachevska Natalya, Farooq Hafsa
Albert Einstein Medical Center
University of Massachusetts Memorial Medical Center
Kikuchi-Fujimoto disease, alternatively referred to as histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease, is a rare yet self-limiting inflammatory condition initially documented by Japanese pathologists Kikuchi and Fujimoto in Japan in 1972. Although the disease primarily affects young and pediatric patients of Asian descent, cases have been documented in individuals of diverse ages and ethnic backgrounds. The typical presentation is acute to subacute, characterized by painful, tender, mobile cervical lymphadenopathy associated with systemic symptoms, including fevers, malaise, weight loss, arthralgias, and various skin manifestations. An excisional lymph node biopsy is imperative for confirming a definitive diagnosis, revealing a deficiency of neutrophils and eosinophils. Immunohistochemistry will demonstrate histiocytes positive for myeloperoxidase and CD68, T cells positive for CD8, and a minimal presence of B cells. Fine-needle aspiration is typically inadequate for confirming the diagnosis due to the limited tissue sample acquired. Distinguishing Kikuchi-Fujimoto disease from lymphomas and infectious etiologies is critical, and additional support for the histologic diagnosis can be obtained through cultures and serological testing. Although the histology in systemic lupus erythematosus (SLE) lymphadenitis may bear a resemblance to Kikuchi-Fujimoto disease, the presence of hematoxylin bodies in SLE lymphadenitis aids in its distinction from Kikuchi-Fujimoto disease. SLE is the most prevalent autoimmune condition associated with Kikuchi-Fujimoto disease. However, unlike SLE, Kikuchi-Fujimoto disease typically follows a self-limiting course lasting several months, with a low recurrence rate of approximately 3% to 4%. The management of Kikuchi-Fujimoto disease primarily involves supportive care for patients, with the use of corticosteroids and immunosuppression reserved for cases of severe or recurrent disease. The prognosis is excellent, with rare complications such as hemophagocytic lymphohistiocytosis (HLH). Diagnostic challenges arise from the rarity of the disease, potentially leading to patients receiving inappropriate treatment for alternative etiologies. Therefore, raising awareness among clinicians and pathologists about this rare condition can significantly improve patient outcomes.
菊池-藤本病，也被称为组织细胞坏死性淋巴结炎或菊池-藤本病，是一种罕见的自限性炎症性疾病，1972年由日本病理学家菊池和藤本在日本首次记录。虽然该疾病主要影响亚洲血统的年轻和儿科患者，但不同年龄和种族背景的个体中均有病例记录。典型表现为急性至亚急性，特征为伴有全身症状（包括发热、不适、体重减轻、关节痛和各种皮肤表现）的疼痛、压痛、可活动的颈部淋巴结病。切除性淋巴结活检对于确诊至关重要，可显示中性粒细胞和嗜酸性粒细胞缺乏。免疫组织化学将显示髓过氧化物酶和CD68阳性的组织细胞、CD8阳性的T细胞以及少量B细胞。由于获取的组织样本有限，细针穿刺通常不足以确诊。将菊池-藤本病与淋巴瘤和感染性病因相鉴别至关重要，可通过培养和血清学检测获得对组织学诊断的额外支持。虽然系统性红斑狼疮（SLE）淋巴结炎的组织学表现可能与菊池-藤本病相似，但SLE淋巴结炎中苏木精小体的存在有助于将其与菊池-藤本病区分开来。SLE是与菊池-藤本病相关的最常见自身免疫性疾病。然而与SLE不同，菊池-藤本病通常呈自限性病程，持续数月，复发率低，约为3%至4%。菊池-藤本病的治疗主要包括对患者的支持治疗，仅在严重或复发病例中使用皮质类固醇和免疫抑制治疗。预后良好，罕见并发症如噬血细胞性淋巴组织细胞增生症（HLH）。由于该疾病罕见，诊断具有挑战性，可能导致患者因其他病因接受不适当的治疗。因此，提高临床医生和病理学家对这种罕见疾病的认识可显著改善患者预后。
