Kikuchi-Fujimoto disease: A comprehensive review

Abstract

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.

Keywords: Cervical lymphadenopathy, Histiocytic necrotizing lymphadenitis, Kikuchi’s disease, Lymphadenopathy, Lymphoma, Systemic lupus erythematosus

Core Tip: Kikuchi-Fujimoto disease or Kikuchi disease described originally in young Japanese women is a rare benign cause of fever and lymphadenopathy usually involving cervical lymph nodes. The disease has been reported worldwide in both genders across ethnic and age groups. Histopathologically, histiocytic necrotizing lymphadenitis is characteristic and needs differentiation from more serious conditions such as malignant lymphoma in acute or subacute form. Its long-term prognosis is favorable albeit long-term follow-up is recommended because of a rare but increased risk of developing systemic lupus erythematosus/other autoimmune disorders. Patients with a severe or recurrent disease need treatment with nonsteroidal anti-inflammatory drugs, systemic corticosteroids, and/or other immunomodulators.