Introduction: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare disease of uncertain origin proposed to be secondary to viral or autoimmune causes. It presents as unilatera...Introduction: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare disease of uncertain origin proposed to be secondary to viral or autoimmune causes. It presents as unilateral cervical lymphadenopathy and fever, typically resolving within a few months, however, it mimics malignant lymphoma, leading to extensive work-up fearing malignancy. Case History: A 33-year-old female with a history of Sjögren’s syndrome, rheumatoid arthritis (not on immunosuppressive therapy) and recent COVID-19 infection two months ago presented with episodes of flu-like symptoms;fever (101˚F), chills, and myalgias for two months. Upon evaluation, she had leukopenia (WBC 1.8 k/uL), neutropenia (1.0 k/uL), elevated CRP (134 mg/L), and CT neck demonstrated multiple enlarged cervical lymph nodes with necrosis. The patient was started on Cefdinir and doxycycline for a possible atypical infection given neutropenic fevers and the patient continued to have fevers. A biopsy showed focal necrosis with no evidence of malignancy, suggesting KFD that was likely triggered by a recent COVID infection. She was treated with Prednisone and naproxen, which led to an improvement in symptoms and recovery of her pancytopenia. Discussion: This case is unique since the patient’s recent infection with COVID-19 may have triggered the clinical manifestations of KFD. There have been a few case reports of children who were diagnosed with KFD after a COVID-19 infection and adults diagnosed with KFD following COVID vaccinations;however, this is the first case report involving a young adult in her thirties who was diagnosed with KFD two months after COVID-19.展开更多
Kikuchi-Fujimoto disease,a rare form of necrotizing lymphadenitis,is an uncommon,benign,self-limiting disorder of obscure etiology.It affects mostly young adults of both genders.Clinically,it presents with fever and l...Kikuchi-Fujimoto disease,a rare form of necrotizing lymphadenitis,is an uncommon,benign,self-limiting disorder of obscure etiology.It affects mostly young adults of both genders.Clinically,it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss,splenomegaly,leucopenia,and elevated erythrocyte sedimentation rate feature in severely affected patients.Cutaneous involvement occurs in about 30%-40%of cases as facial erythema and nonspecific erythematous papules,plaques,acneiform or morbilliform lesions of great histologic heterogeneity.Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede,develop subsequently,or sometimes be associated concurrently with Kikuchi-Fujimoto disease.It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis,cat-scratch disease,Sweet’s syndrome,Still’s disease,drug eruptions,infectious mononucleosis,and viral or tubercular lymphadenitis are other common differentials.Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value.Since its diagnosis is exclusively from histopathology,it needs to be evaluated more carefully;an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials.Its treatment with systemic corticosteroids,hydroxychloroquine,or antimicrobial agents mostly remains empirical.The article reviews clinicoepidemiological,diagnostic,and management aspects of KFD from the perspective of practicing clinicians.展开更多
文摘Introduction: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare disease of uncertain origin proposed to be secondary to viral or autoimmune causes. It presents as unilateral cervical lymphadenopathy and fever, typically resolving within a few months, however, it mimics malignant lymphoma, leading to extensive work-up fearing malignancy. Case History: A 33-year-old female with a history of Sjögren’s syndrome, rheumatoid arthritis (not on immunosuppressive therapy) and recent COVID-19 infection two months ago presented with episodes of flu-like symptoms;fever (101˚F), chills, and myalgias for two months. Upon evaluation, she had leukopenia (WBC 1.8 k/uL), neutropenia (1.0 k/uL), elevated CRP (134 mg/L), and CT neck demonstrated multiple enlarged cervical lymph nodes with necrosis. The patient was started on Cefdinir and doxycycline for a possible atypical infection given neutropenic fevers and the patient continued to have fevers. A biopsy showed focal necrosis with no evidence of malignancy, suggesting KFD that was likely triggered by a recent COVID infection. She was treated with Prednisone and naproxen, which led to an improvement in symptoms and recovery of her pancytopenia. Discussion: This case is unique since the patient’s recent infection with COVID-19 may have triggered the clinical manifestations of KFD. There have been a few case reports of children who were diagnosed with KFD after a COVID-19 infection and adults diagnosed with KFD following COVID vaccinations;however, this is the first case report involving a young adult in her thirties who was diagnosed with KFD two months after COVID-19.
文摘Kikuchi-Fujimoto disease,a rare form of necrotizing lymphadenitis,is an uncommon,benign,self-limiting disorder of obscure etiology.It affects mostly young adults of both genders.Clinically,it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss,splenomegaly,leucopenia,and elevated erythrocyte sedimentation rate feature in severely affected patients.Cutaneous involvement occurs in about 30%-40%of cases as facial erythema and nonspecific erythematous papules,plaques,acneiform or morbilliform lesions of great histologic heterogeneity.Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede,develop subsequently,or sometimes be associated concurrently with Kikuchi-Fujimoto disease.It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis,cat-scratch disease,Sweet’s syndrome,Still’s disease,drug eruptions,infectious mononucleosis,and viral or tubercular lymphadenitis are other common differentials.Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value.Since its diagnosis is exclusively from histopathology,it needs to be evaluated more carefully;an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials.Its treatment with systemic corticosteroids,hydroxychloroquine,or antimicrobial agents mostly remains empirical.The article reviews clinicoepidemiological,diagnostic,and management aspects of KFD from the perspective of practicing clinicians.
