Kikuchi–Fujimoto disease associated with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report

Abstract

Background Kikuchi–Fujimoto disease, which was originally described in young women, is a benign condition characterized by necrotizing lymphadenitis and fever. Even though the clinical course is usually self-limiting, it can be associated with recurrences and rarely can be associated with systemic lupus erythematosus or can be complicated with hemophagocytic lymphohistiocytosis. We report the case of a 17-year-old Sri Lankan Sinhalese schoolboy who presented with fever and cervical lymphadenopathy diagnosed as Kikuchi–Fujimoto disease and was complicated with hemophagocytic lymphohistiocytosis subsequently. Later he fulfilled the criteria for systemic lupus erythematosus. Case presentation A 17-year-old previously healthy Sinhalese schoolboy presented with high-grade fever associated with chills and rigors associated with loss of appetite and loss of weight for more than 40 days. On examination, he had bilateral firm matted tender cervical lymphadenopathy and firm hepatomegaly. An excision biopsy of his right cervical lymph node revealed necrotizing lymphadenitis and immunohistochemistry of a lymph node biopsy favored Kikuchi disease. Initial antinuclear antibody and anti-double-stranded deoxyribonucleic acid tests were negative and his C3 and C4 levels were normal. An infections screening was negative. He was treated with steroids. While in hospital he developed hemophagocytic lymphohistiocytosis and renal impairment. Later his antinuclear antibody titer became positive in 1:160 and fulfilled the diagnostic criteria for systemic lupus erythematosus. He was managed with steroids and immune suppressive drugs and showed remarkable improvement. Conclusion Although Kikuchi–Fujimoto disease is uncommon in male patients, it needs to be considered in patients with lymphadenopathy and fever. The disease can be complicated with hemophagocytic lymphohistiocytosis and the patients need continuous monitoring for the possible development of systemic lupus erythematosus later in the course.

Kikuchi Fujimoto disease: sinister presentation, good prognosis

“…The most common associated autoimmune disease in KFD is SLE [14]. KFD can precede SLE or present simultaneously [15]. Some studies suggest screening for SLE with an antinuclear antibody test during the initial diagnostic work up, especially in patients with cutaneous involvement [15].…”

“…KFD can precede SLE or present simultaneously [15]. Some studies suggest screening for SLE with an antinuclear antibody test during the initial diagnostic work up, especially in patients with cutaneous involvement [15]. Some studies believe KFD may represent a self-limiting lupus-like spectrum induced by lymphocytes [6,16].…”

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

“…This introduces the question of whether patients with diagnosed KFD require periodical serological or clinical follow‐up for the development of SLE. Some authors do recommend follow up, especially for patients who develop severe complications 24 or those with cutaneous manifestations 25,26 …”

“…1 This introduces the question of whether patients with diagnosed KFD require periodical serological or clinical follow-up for the development of SLE. Some authors do recommend follow up, especially for patients who develop severe complications 24 or those with cutaneous manifestations. 25,26 Although the association is unclear, no guidelines have been set on the follow up of patients with Kikuchi, and no indications for rheumatology referral have been established.…”

Kikuchi-Fujimoto Disease with Hemophagocytic Syndrome and Pleural Effusion in an Elder Man: A Case Report and Literature Review

“…The pathogenesis of KFD, may involve the activation of CD8+ T cell, leads to T cell proliferation and dying via the Fas and perforin pathways; then, macrophages phagocytic apoptotic cells, and resulting in typical necrotizing lymphadenitis [10]. However, in a meta-analysis using 41 studies, [15]. Since Most cases were showed KFD can occur before, after or concurrently with SLE, particular in these share same characteristics: child-bearing period female, fever, lymphadenopathy, cytopenia, arthralgia and skin lesions [14]- [19].…”