Abstract
Kikuchi-Fujimoto disease (KFD) is a rare, benign and usually self-limiting disorder that more often affects young women, which is characterized by cervical lymphadenopathy and fever. Clinical presentation may be indistinguishable from other diseases, and its inclusion in the differential diagnosis of lymphoproliferative, infective and autoimmune diseases is essential. An association with systemic lupus erythematosus is acknowledged. We present 2 different cases of 2 young women with KFD; the first case highlights the classic diagnostic features of this rare entity, and the second, the findings when KFD occurs in association with systemic lupus erythematosus. LEARNING POINTS: Fever and cervical lymphadenopathy presenting in a young woman are the main features of KFD.A timely excisional lymph node biopsy is of the utmost importance in establishing a correct diagnosis and in the management of this condition.In most patients with KFD the course of the disease is benign and self-limiting; however, when associated with other conditions (mainly systemic lupus erythematosus) it can follow a more severe evolution.