Cervical Lymphadenopathy in Two Young Women: The Same Rare D
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Fever and cervical lymphadenopathy presenting in a young woman are the main features of Kikuchi-Fujimoto disease (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.

KFD was first described in Japan almost simultaneously by Kikuchi and by Fujimoto and colleagues in 1972. KFD is an extremely rare disease and although it can affect both sexes and all age groups, it occurs more often in young women (<40 years of age).

The precise etiology remains unknown, although several types of infection have been hypothesized to act as causal agents. Some authors have proposed local hyperimmune stimulation after infection, influenced by individual genetic predisposition or autoimmune mechanisms, as a possible pathogenic feature. KFD frequently manifests as an acute or subacute illness, evolving over a period of 2 to 3 weeks.

Cervical lymphadenopathies (70–100%), usually unilateral and tender, and fever (30–50%) are the most common symptoms in KFD patients. Less commonly, patients can present with weight loss, night sweats, asthenia, arthralgias, cutaneous manifestations or hepatosplenomegaly. Rarely, generalized lymphadenopathies can occur (5% of cases).
Laboratory tests are frequently unremarkable; however, mild leucopenia, anemia and, more rarely, thrombocytopenia can be found, as well as elevated inflammatory markers (CRP and ESR).

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. This report presents 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.

Source: https://www.ejcrim.com/index.php/EJCRIM/article/view/1516/1991
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