A0050 - Disappearing Act: A Case of Kikuchi-Fujimoto Disease

Steve D'Souza, MD, Buyng Soo Yoo, MD, Parth Parekh, MD
Eastern Virginia Medical School, Norfolk, VA

Introduction: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limited disorder that most frequently presents with tender lymphadenopathy, rash, fever, and night sweats. We present a case of a patient with diffuse lymphadenopathy and fever diagnosed with KFD.

Case Description/Methods: A 31-year-old female without significant past medical history presented for evaluation for three weeks of dull abdominal pain, fevers, nausea, non-bloody emesis, and 14lb weight loss. Outpatient workup of diffuse lymphadenopathy by her primary care physician with CT and subsequent MRI of abdomen and pelvis had demonstrated a bilobed retroperitoneal mass adjacent to the pancreatic head process concerning for necrotic lymph nodes versus malignancy. Autoimmune serologies were negative, and culture data and viral serologies were negative. She underwent endoscopic ultrasound and fine needle aspiration of the mass. Cytology demonstrated benign-appearing ductal epithelial cells, necrotic debris, and spindle cells with fibro-inflammatory stroma, and was negative for malignancy. Flow cytometry was also negative for malignancy. She continued to remain febrile and was placed on antibiotics following consultation with the infectious disease service. Repeat biopsy was discussed with interventional radiology and general surgery services; however, there lacked an adequate window for percutaneous or laparoscopic approaches. The patient's symptoms were controlled with antipyretics and antiemetics and she was discharged on a course of oral antibiotics. Repeat endoscopic ultrasound-guided biopsy was performed two months later of an enlarged peripancreatic lymph node. Cytology revealed mixed T and B cells with zones of necrosis, but no granulomas. No features of malignancy were seen, and fungal and acid-fast staining were negative. The patient was closely monitored, and her adenopathy and symptoms completely resolved two months later.

Discussion: The diagnosis of KFD is based on histologic examination which demonstrates proliferative and necrotizing lymph nodes with abundant T cells in the lesion. The disease is benign and self-limited, with primary treatment being supportive care, and it has a low recurrence rate. Due to its rarity, the disease poses a diagnostic challenge to clinicians, as it often mimics systemic lupus erythematosus and non-Hodgkin’s lymphoma. Our patient presented with fever, weight loss, and a retroperitoneal mass concerning for malignancy, that was found to be KFD.



Disclosures:


Steve D'Souza, MD, Buyng Soo Yoo, MD, Parth Parekh, MD. A0050 - Disappearing Act: A Case of Kikuchi-Fujimoto Disease, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.