Kikuchi-Fujimoto disease.

Diagnosis

Kikuchi-Fujimoto disease.

Diagnosis

Kikuchi-Fujimoto disease.

History

17-year-old girl with tender cervical lymph nodes and fever . No weight loss. No skin rash.

Findings

FDG PET/CT demonstrates clusters of small lymph nodes , up to 12 mm with uptake up to 7.1 SUV max.
No frank conglomerate.
No evidence of distant disease, especially in the mediastinum and no evidence of extranodal uptake.

Discussion

Kikuchi-Fujimoto Disease (KFD) is rare affection usually found in young Asiatic patients in the first five decades with a 4:1 female to male ratio.
It is however infrequent in children. It has been associated to systemic lupus erythematosus (SLE) either during the course of the disease or as a harbinger .
Clinical presentation is the presence of tender cervical adenopathies with fever and weight loss. Skin rash is found in up to 40% of patients.
Other KFD locations, mimicking aggressive disease have been reported. Diagnosis is challenging in SLE patients as active lymph nodes are demonstrated in various rheumatologic pathologies and as both lymphoma and KFD can occur in SLE patients.
FDG PET/CT demonstrate clusters of smaller lymph nodes when compared to lymphoma but with intense FDG uptake, increasing with time, making the differentiation between both diseases difficult . In addition, FDG PET/CT identifies additional lymph nodes sites non suspected clinically. However, no extranodal lesions have been identified with FDG PET/CT in patients with KFD.
Pathology shows subacute necrotizing lymphadenitis, CD68 histiocytic cells, CD8 T cells but no follicular hyperplasia or neutrophils.
The etiology is unknown but likely autoimmune. Treatment options includes analgesics, antipyretics, steroids, minoclyclin, hydroxycloroquine and intravenous immunoglobulins.

Key points

Differential Diagnosis:
• Lymphoma,
• Metastatic disease
• Benign lymphoproliferative diseases such as Castleman,. • Infection: Epstein-Barr, mycobacteria, Brucellosis, Histoplasmosis, acute lymphadenitis. • Histiocytosis: Langerhans cell histiocytosis, Rosai-Dorfman. • Systemic Lupus Erythematosus (SLE)

References

Necrotizing Lymphadenitis (Kikuchi`s Disease ). HL Smith II. Pediatrics 1993;91:152
Kikuchi–Fujimoto disease: a diagnostic dilemma. LM Mathew. Int J Dermatol 2016;55:1069-1075Kikuchi Disease. Masab M . StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020-.2020 Apr 8.La maladie de Kikuchi-Fujimoto ; un diagnostic différentiel méconnu du lymphome chez l’adolescent. Kikuchi-Fujimoto disease mimicking malignant lymphoma inadolescents. A. Escudier et al. Arch Pédiatr 2017;24:471-475Kikuchi Disease mimicking Lymphadenopathy on F18 FDG PET/CT in patients with breast cancer. JP Hwang. Nucl Med Mol Imaging 2015;49:167-168Systemic Lupus Erythematosus Associated Pitfalls on 18F-FDG PET/CT: Reactive Follicular Hyperplasia, Kikuchi-Fujimoto Disease, Inflammation and Lymphoid Hyperplasia of the Spleen Mimicking Lymphoma. W Makis et al. Nucl Med Mol Imaging 2018;52:74-79Kikuchi-Fujimoto Disease. PET/CT Assessment of a Rare Cause of Cervical Lymphadenopathy, T Tsujikawa et al. Clin Nucl Med 2011;36:661-664Kikuchi-Fujimoto Disease with 18F-Fludeoxyglucose Uptake in Cervical Lymph Nodes on Dual-time-point Imaging Positron Emission Tomography/Computed Tomography Mimicking Malignant Disease. Aoyama KI et al. World J Nucl Med 2017;16:68-70Kikuchi-Fujimoto disease mimicking malignant lymphoma with 2-[18F]fluoro-2-deoxy-D-glucose PET/CT in children. JE Kim. Korean J Pediatr 2014;57:226-231