Kikuchi-Fujimoto Disease: An Atypical Presentation of a Rare Disease

Moyer, Amanda; Hanafi, Muhammad Z.; Scordino, Teresa; Bronze, Michael S.

doi:10.7759/cureus.3999

Cited by 6 publications

(8 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Fragility of superficial capillaries due to retinal vasculitis (although fluorescein angiography (FA) was not performed in this patient) should also be taken into consideration as probable mechanisms. 4 , 55 …”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Bilateral preretinal hemorrhage associated with Kikuchi-Fujimoto disease

Akhavanrezayat

Cooper

Hassan

et al. 2021

American Journal of Ophthalmology Case Reports

View full text Add to dashboard Cite

Purpose To present a case of a patient with human immunodeficiency virus (HIV) disease and Kikuchi-Fujimoto disease (KFD) who presented with a unique pattern of retinopathy. Observations A 7-year-old Taiwanese girl with HIV disease who was recently diagnosed with KFD had a sudden onset of blurry vision in both eyes one month after her KFD systemic symptoms had relatively resolved. Ophthalmic examination showed decreased visual acuity in both eyes (OU). On fundus examination, she had bilateral preretinal, subhyaloid, and vitreous hemorrhage that was more severe than anemic retinopathy. Conclusion Ocular manifestations in Kikuchi-Fujimoto disease are rare; however, if they occur, presentations may vary. The exact etiology of the disease has remained elusive and controversial. This case is the first report of a patient with HIV disease and KFD presenting with ocular involvement. Furthermore, bilateral preretinal, subhyaloid, and vitreous hemorrhage, which was beyond anemic retinopathy, is an unprecedented manifestation of KFD that has not been previously reported. This case highlights the necessity for clinicians to consider all possible differential diagnoses when evaluating patients with similar findings to identify the best therapeutic approach and avoid unnecessary treatment.

show abstract

Section: Discussionmentioning

confidence: 99%

“…3 Due to its usually benign course and self-limited nature, KFD tends to subside in a few months, and patients with KFD are often observed. 4 , 5 …”

Section: Introductionmentioning

confidence: 99%

Bilateral preretinal hemorrhage associated with Kikuchi-Fujimoto disease

Akhavanrezayat

Cooper

Hassan

et al. 2021

American Journal of Ophthalmology Case Reports

View full text Add to dashboard Cite

show abstract

“…23 Kikuchi may be a self-limited SLE-like condition or mimic, which may be secondary to virally infected lymphocytes. 6 Additionally, Kikuchi may be a clinical or histopathological phase of lupus lymphadenitis. 20 On the contrary, many reported cases had no association with SLE.…”

Section: Discussionmentioning

confidence: 99%

An atypical presentation of Kikuchi‐Fujimoto disease: A case report & literature review

Halawa

Ahmad

Nashwan

2020

Clinical Case Reports

View full text Add to dashboard Cite

BACKGROUND Kikuchi-Fujimoto disease (KFD) is a self-limiting condition that typically presents with lymphadenopathy and fever. It is autoimmune in nature and possibly associated with systemic lupus erythematosus (SLE). The case report highlights an unusual case of KFD associated with autoimmune hemolytic anemia and provided a review for other atypical presentations. Kikuchi Disease, also known as Kikuchi-Fujimoto disease (KFD), is a benign, self-limiting condition of undetermined etiology, that commonly presents with lymphadenopathy and fever. The disease was initially described in young women from Japan. However, it became more apparent that although Kikuchi is frequently reported in Asia, it does occur in various racial groups. In a retrospective literature review by Kucukardali et al, 1 cases were reported from the USA, Taiwan, and Spain, with a male to female ratio of 1:3. Although the pathogenesis is not completely understood, the underlying mechanism is thought to be an immune response to an infectious agent, with some studies reporting Epstein-Barr virus (EBV), 2 Human herpesvirus 6 (HHV6), 3 parvovirus, 4 and others reporting bacterial and parasitic infection. The immune response predominantly involves T cells and histiocytes, in addition to elevated levels of cytokines, markedly interferon-gamma and IL-6. 5 The most common presentation in the majority of cases of KFD is cervical lymphadenopathy. In a literature review by Kucukardali et al, 1 out of 244 cases of KFD, 100% had cervical lymphadenopathy, and 35% of cases complained of fever. Other reported signs and symptoms include rash, arthritis, and hepatosplenomegaly.

show abstract

“…Histiocytic necrotizing lymphadenitis was first described by Kikuchi and separately by Fujimoto, Kojima, and Yamaguchi in 1972. 12 This self-limiting condition is characterized by elevated acute phase reactants and leukopenia or lymphopenia. 5 Cutaneous manifestations are reported in 16%–40% of patients, typically revealing histiocytic aggregates, atypical lymphoid cells, karyorrhectic debris, and patchy necrosis.…”

Section: Discussionmentioning

confidence: 99%

“…5 Cutaneous manifestations are reported in 16%–40% of patients, typically revealing histiocytic aggregates, atypical lymphoid cells, karyorrhectic debris, and patchy necrosis. 12 KFD typically resolves in 1–4 months without specific treatment. 5 , 13 However, in severe cases, successful treatment has been reported with corticosteroids and intravenous immunoglobulin.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of a unilateral neck mass in a 16-year-old female: Kikuchi-Fujimoto disease with superimposed bacterial lymphadenitis

Burns

Pathania

Griesser

et al. 2020

SAGE Open Medical Case Reports

View full text Add to dashboard Cite

The evaluation of pediatric neck masses offers the opportunity for consideration of a diverse range of pathologies, from infectious to neoplastic. A 16-year-old female presented with 2 weeks of worsening swelling and pain of a left-sided neck mass. Findings were consistent with Epstein–Barr virus and cytomegalovirus coinfection, but considering profound lymphadenopathy of the supraclavicular, mammillary, and axillary chains, further investigations were undertaken. Hematopathologic examination demonstrated necrotizing lymphadenitis, consistent with Kikuchi-Fujimoto disease. A diagnosis of Kikuchi-Fujimoto disease alongside chronic bacterial lymphadenitis was made on the basis of her response to clindamycin, and the chronic course of her illness and subsequent persistence of the swelling managed on an outpatient basis. The case study describes the initial diagnostic considerations and management as well as a review of the disease pathology.

show abstract

Kikuchi-Fujimoto Disease: An Atypical Presentation of a Rare Disease

Cited by 6 publications

References 16 publications

Bilateral preretinal hemorrhage associated with Kikuchi-Fujimoto disease

Bilateral preretinal hemorrhage associated with Kikuchi-Fujimoto disease

An atypical presentation of Kikuchi‐Fujimoto disease: A case report & literature review

Evaluation of a unilateral neck mass in a 16-year-old female: Kikuchi-Fujimoto disease with superimposed bacterial lymphadenitis

Contact Info

Product

Resources

About