Necrotizing cervical lymphadenopathy: Kikuchi-Fujimoto disease in a young male

Abstract

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare benign disease, more common in Japanese women, with only a few cases reported from other parts of the world. The pathogenesis is poorly understood. A 22-year-old man noticed a node growth a month before his admittance. At first he noticed one enlarged node in the left axilla, later also the nodes on his neck and in his right axilla. Before that he had felt shivery and reported night sweats. Physical examination revealed bilateral enlarged (up to 2 cm) painful but not inflamed lymph nodes on the neck and in both axillae. The patient was diagnosed with KFD on histological examination of the nodes and on the basis of typical changes. No medical treatment was prescribed and the patient has been without symptoms for the past two years. The clinical picture of KFD is nonspecific and variable. Most patients complain of enlarged cervical nodes (up to 2 cm) accompanied by various symptoms (fever, malaise, night sweats, weight loss). The enlarged nodes, which are mobile, rarely appear in other locations. The only way of proving whether the disease is present or not is by histological examination of the node, which may show the following changes typical for KFD: paracortical necrotic foci, surrounded by histiocytic aggregates, irregular rounded eosinophilic areas of different sizes in the paracortex and cortex with the presence of numerous histiocytes, lymphocytes, immunoblasts, plasmacytoid monocytes and eosinophilic granulated cellular debris. Histiocytes had mostly turned into foamy macrophages. It is typical not to find epithelioid cells and neutrophil granulocytes in the necrotic centers. In particular, the disease needs to be distinguished from high-grade lymphoma and SLE lymphadenitis. There is no specific medical treatment for KFD since the disease usually resolves spontaneously over four months. Disease recurrence or fatalities are unusual.