Histiocytic Necrotizing Lymphadenitis or Kikuchi–Fujimoto Disease: Clinical Experiences in North Africa

Introduction: Kikuchi-Fujimoto disease or Kikuchi disease (KD) or histiocytic necrotizing lymphadenitis is an uncommon disease more frequent in Asia. It is diagnosed with lymph node histopathological findings.

Objective: To perform a systematic review of Kikuchi’s disease (KD) cases and describe clinical manifestations and associated etiology in the Tunisian population.

Methods: We conducted a multicentric descriptive study collecting patients with histologically proven KD. Clinical data, laboratory results, histopathologic examination, associated pathologies, and patient evolution were included.

Results: The search included 9 patients. They were 7 women and 2 males with a median age of 45 years old [24-72]. Common clinical manifestations were fever (n=8) and arthralgia (n=7). Lymph nodes mainly were bilateral (n=8), multiple (n=9), and in the cervical region (n=8) and axillary area (n=6). Laboratory results showed inflammatory syndrome and elevated lactate dehydrogenase. Areas of necrosis, karyorrhectic debris, and accumulation of histiocytes were specific histologic findings noted for all patients. Systemic Lupus Erythematosus was associated with KD in 2 patients and with Sjögren disease in one of them. Other associated pathologies were Lymphohistiocytic activation syndrome, lymphocytic meningitis, sepsis from a urinary tract infection, and idiopathic interstitial lung disease. Corticosteroids were prescribed in 4 cases. The other 5 cases had spontaneous regression of symptoms. The outcomes were favorable with recovery in 3 cases. Five patients developed Hodgkin lymphoma, myelodysplastic syndrome, and lupus nephropathy after KD diagnosis.

Conclusions: KD is exceptional in North Africa. The study noted the same clinical and histological findings in the literature. SLE was the most associated pathology. No ethnic variability was detected.