Abstract

KIKUCHI-FUJIMOTO DISEASE (HISTIOCYTIC NECROTIZING LYMPHADENITIS): A CASE REPORT

Kikuchi-Fujimoto disease (KFD) is a self-limited pathological entity that is benign in its course. The main features of this disease are tender regional cervical lymphadenopathy, usually accompanied by low-grade fever and night sweats. Less frequently patients may report weight loss, nausea, vomiting, and sore throat. Often referred to as KFD; Kikuchi-Fujimoto disease has a global prevalence with high report rates from Japan and other Asian regions. That said, it is a very uncommon case to encounter due to its rare occurrence. A viral causation is suggested keeping in view the clinical presentation, the histology and immunohistochemical pattern. The recommended procedure to diagnose this disease is an excisional biopsy of an affected lymph node. This histopathologic analysis is essential for the clinician to differentiate it from similarly presenting conditions like tuberculous lymphadenitis (scrofula), lupus (SLE) or malignant lymphoma to state a few. This is especially pertinent in regions where there is a high index of suspicion for tuberculosis given its high prevalence, like Pakistan and India for example. It is also essential to inculcate an understanding of this clinically and histopathologically challenging disease amongst physicians and pathologists alike to decrease the risk of misdiagnosis. Steps to increase awareness will also help curb the excessive costs and unnecessary interventions that go with wrong diagnoses. In young patients who present with lymph node enlargement in the posterior cervical chain; a biopsied node showing cellular and nuclear fragmentation along with features of necrosis should incite the consideration of KFD amongst other differentials. Once diagnosed; symptomatic treatment with NSAIDs, analgesics, antipyretics and in some cases corticosteroids is sufficient as the self limited disease resolves by itself within a period of 1 month in most cases. It is unlikely to stretch beyond 4 months. Patients with KFD are followed for many years nonetheless, since these patients are at a slightly increased risk of developing systemic lupus erythmatosus later in life than the general population. The case we report is that of an 18 year old female patient who presented with a month history of low grade fever, night sweats and cervical lymphadenopathy, which was minimally tender. She was initially labeled as a case of tuberculous lymphadenitis and put on antituberculous therapy. However, she did not respond and further diagnostic studies revealed the presence of KFD.

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