Kikuchi disease, also known as histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease, is a rare but self-limiting inflammatory condition. The condition was initially identified by Japanese pathologists Kikuchi and Fujimoto in Japan in 1972 [1].  The precise etiology remains uncertain, though infection and autoimmunity have been postulated as potential causes. Lymphadenopathy frequently abates within a few weeks to six months. Recurrence rates may reach 3% [2]. Clinical manifestations may resemble tuberculosis and lymphoma. This case study presents a 22-year-old female with cervical lymphadenopathy. Diagnosis was made by histopathology, and the patient recovered following treatment.

  A 22-year-old female presented with a complaint of a lump on the upper neck, in close proximity to the lower jaw, that had manifested one month prior to admission. Initially, the lump was approximately the size of a marble, but it subsequently increased in size, reaching the dimensions of a chicken egg. The patient denied any history of weight loss, joint pain, nausea, vomiting, diarrhea, or night sweats. The patient had previously been treated at the health center with antibiotics, but this course of treatment did not result in an improvement in the patient's condition. Upon physical examination, the patient's blood pressure was 120/70 mmHg, pulse 75x/min, temperature 37°C, and a palpable mass of spongy consistency and mobile size 5 was observed in the region of the left colli level IA-B. The mass was 5 cm in diameter, exhibited a warm sensation, and had a color and consistency similar to that of the surrounding skin. It was located in the region of the left submandibular gland and was mobile, measuring 2 x 3 cm. There were no abnormalities in the examination of the heart, lungs, or nerves. Routine blood tests, blood glucose, hepatitis antigen, bleeding time, and the clotting time, did not reveal any abnormalities (Table 1). 

Table 1. Laboratory results performed on the patient

The patient underwent neck ultrasound examination with results of submandibular lymphadenopathy and left colli (Figure 1). Excision surgery and histopathological examination were performed with the results of histiocytic necrotizing lymphadenitis (Figure 2A and B). The patient was treated for 2 days with analgesics and antibiotics. Patient control 1 week later.

Figure 1. Submandibular and left colli lymphadenopathy with normal thyroid.

Figure 2. Lymph node histopathology in Kikuchi-Fujimoto disease. A. Macroscopic view of the preparation taken by excisional biopsy. B. Areas of histiocytic infiltration and karyorrhexis debris.

Kikuchi Fujimoto disease (KFD) is a rare condition that presents with regional necrotizing subacute lymphadenopathy. KFD is a benign and self-limiting condition that often presents with mild febrile symptoms and some systemic manifestations. The condition was first identified by Japanese pathologists Kikuchi and Fujimoto in Japan in 1972 [1,3]. KFD has been documented in both pediatric and adult patients across the globe, including in Western and Asian populations, with a notably elevated prevalence in Japan. The condition is more prevalent in females than in males, with a peak incidence between the ages of 20 and 35. The ratio of female to male cases is approximately 2:1 [3,4]. The precise etiology remains unknown, although several potential agents have been identified as possible triggers, including Epstein-Barr Virus (EBV), Yersinia enterocolitica, Toxoplasma, and Streptococcus pneumoniae. Additionally, there have been reports of an association between KFD and Systemic Lupus Erythematosus (SLE) [3], with up to 25% of patients with KFD eventually developing SLE [5]. 

The onset of KFD may be acute or subacute, with a potential for spontaneous resolution within a few weeks. KFD typically manifests as posterior cervical lymphadenopathy (in 60% to 90% of cases), frequently accompanied by involvement of the axillary or supraclavicular lymph nodes. In addition to lymphadenopathy, the syndrome is frequently accompanied by fever (in 35% to 77% of cases) and, less commonly, by weight loss, nausea and vomiting, weakness, dizziness, arthralgia, night sweats, symptoms of an upper respiratory tract infection, and throat pain [6]. In most cases, the lymphadenopathy associated with KFD is less than 3 cm in size; however, some reports indicate that it can reach up to 5 to 6 cm. The characteristics of lymphadenopathy associated with KFD are typically described as mobile, dense, with regular margins, discrete, and accompanied by pain [3]. The duration of fever is variable, lasting from one to seven weeks, with a temperature between 38.6°C and 40.5°C. Lymph node tenderness on palpation may or may not be present. It has been reported that more than 30% of patients with KFD have skin complaints such as acneiform eruptions, focal erythema, papules, purpura, or nodules. The presence of skin complaints indicates a severe clinical presentation [7].

The differential diagnosis of KFD includes lymphadenitis, SLE, TB, and lymphoma [6,8]. Infectious causes of lymphadenopathy include airway infections, ear infections, and other foci of infection [9]. Systemic lupus erythematosus (SLE) represents a significant differential diagnosis for fibromuscular dysplasia (FMD). Distinguishing features include the presence of hematoxylin bodies in SLE, which are absent in FMD (10). The gold standard for diagnosing KFD is a histopathologic examination of biopsy preparations. The anatomical pathology of KFD can be classified into three main categories: proliferative, necrotizing, and xanthomatous. The necrotizing type is the most prevalent and is defined by the absence of neutrophil infiltrates, which are surrounded by mononuclear cells and karyorrhectic debris. The proliferative type is characterized by follicular and paracortical hyperplasia with apoptosis. The xanthomatous type is defined by the presence of foamy histiocytes, which represent the healing phase of KFD [3].

Kikuchi-Fujimoto disease is a rare lymphadenopathy that is established by histopathologic examination. Early diagnosis and treatment can help avoid unnecessary examinations and inappropriate treatment of patients.

Conflict of Interest: The authors declare that they have no conflict of interest

Funding: No funding sources

Ethical approval: The study was approved by the YARSI University.

1. Gurung, S., et al. "Kikuchi–Fujimoto Disease in a 20-Year-Old Female: A Case Report." Annals of Medicine & Surgery, vol. 85, no. 5, May 2023, pp. 1894–96.

2. Yoo, I. H., et al. "Recurrent Lymphadenopathy in Children with Kikuchi-Fujimoto Disease." European Journal of Pediatrics, vol. 173, no. 9, 1 Sept. 2014, pp. 1193–99.

3. "Kikuchi-Fujimoto Disease: A Comprehensive Review."

4. Deaver, D., et al. Cancer Control 313 314 Cancer Control [Internet], vol. 21, 2014, www.damloedits.com.

5. Kim, H. Y., et al. "Clinical and Laboratory Characteristics of Kikuchi-Fujimoto Disease According to Age." Frontiers in Pediatrics, 2 Nov. 2021, vol. 9.

6. Perry, A. M., and S. M. Choi. "Kikuchi-Fujimoto Disease: A Review." Archives of Pathology and Laboratory Medicine, College of American Pathologists, 2018, pp. 1341–46.

7. Singh, J. M., and C. B. Shermetaro. "Kikuchi-Fujimoto Disease in Michigan: A Rare Case Report and Review of the Literature." Clinical Medicine Insights: Ear, Nose & Throat, vol. 12, Jan. 2019, 117955061982868.

8. Feder, H. M., et al. "Kikuchi Disease in Connecticut." Journal of Pediatrics, vol. 164, no. 1, 2014.

9. Lelii, M., et al. "Kikuchi-Fujimoto Disease in Children: Two Case Reports and a Review of the Literature." Italian Journal of Pediatrics, 18 July 2018, vol. 44, no. 1.

10. Zuckerman, R., et al. "Persistent Cervical Lymphadenitis in a Patient with Prior Thyroid Cancer Attributed to Kikuchi-Fujimoto Disease." BMJ Case Reports, 21 Oct. 2018, 2018.