Relapsing Kikuchi-Fujimoto Disease With Hemophagocytic Lymphohistiocytosis

Abstract

Kikuchi-Fujimoto disease is a rare, benign, and self-limited disease of uncertain etiology, affecting mostly young female patients. It usually manifests as posterior cervical lymphadenopathy and fever. Its diagnosis is based on typical histopathological changes after the exclusion of other diseases such as lupus, lymphoma, or infectious lymphadenitis. The authors present a 47-year-old female patient with recurring episodes of high fever, urticarial rash, myalgia, arthralgia, fatigue, sore throat, and generalized lymphadenopathy. Blood tests showed increased inflammatory parameters and positive antinuclear antibodies. In the two times the patient was admitted to the hospital there were no infectious agents isolated. The patient didn't fulfill the criteria for diagnosis of lupus or any other autoimmune disease and there was also no evidence of lymphoma or other neoplastic diseases. A positron emission tomography/computed tomography (PET/CT) was performed at the first and second hospitalizations, showing generalized lymphadenopathy. At the first hospitalization, a mediastinal lymph node biopsy was obtained, excluding lymphoproliferative or granulomatous disease. During the course of the second hospitalization, a cervical lymph node was excised for biopsy, the histopathological changes of which suggested the diagnosis of Kikuchi-Fujimoto disease. The clinical course was complicated by hemophagocytic lymphohistiocytosis, with a significant increase in inflammatory markers and liver dysfunction. The patient was treated with prednisolone 1 mg/kg/day, with complete resolution of clinical and biochemical changes.

Keywords: cervical lymphadenopathy; fever; hemophagocytic lymphohistiocytosis; histiocytic necrotizing lymphadenitis; kikuchi-fujimoto disease; migratory arthralgia; urticarial rash.

Figure 1. Patient’s right superior limb showing small erythematous papules coalescing to form plaques predominantly in hand and wrist (dashed arrow). In the forearm region, there is an erythematous patch (normal arrow).

Figure 2. Patient’s right and left lower limbs and part of the left hand. The thighs show symmetrical, prominent erythematous plaques that had started as papules but have coalesced. Some papules and patches in the left hand are also visible.