Kikuchi–Fujimoto disease in the Eastern Mediterranean zone

Abstract

Kikuchi–Fujimoto disease (KFD) is a rare benign and self-limiting syndrome. We aim to review cases of KFD at our institution as a rare illness in the Arab ethnic descent and to analyse reports from most countries in the East Mediterranean zone. This is a retrospective study in which the histopathology database was searched for the diagnosis of KFD. A full review of KFD patients’ medical records was done. Data regarding demographic features, clinical presentation, laboratory findings, comorbidities, and management protocols were obtained. Published KFD cases from east Mediterranean countries were discussed and compared to other parts of the world. Out of 1968 lymph node biopsies studied, 11 (0.6%) cases of KFD were identified. The mean age of patients with KFD was 32 years (4–59). 73% (8/11) were females. The disease was self-limiting in 5 patients (45%); corticosteroid therapy was needed in 4 patients (34%). One patient was treated with methotrexate and one with antibiotics. One patient died as a consequence of lymphoma. Jordanians and Mediterranean populations, especially those of Arab ethnic background, seem to have low rates of KFD. The genetic susceptibility theory may help to explain the significantly higher disease prevalence among East Asians. Early diagnosis of KFD—although challenging—is essential to reduce the morbidity related to this illness.

Drug rash with eosinophilia and systemic symptoms syndrome masquerading as a lymphoproliferative disorder in a young adult on immunosuppressive therapy for rheumatoid arthritis: a case report

“…Hypereosinophilic syndromes are defined by eosinophilia > 1.5 × 10 9 cells/L for > 6 consecutive months, eosinophil-induced organ damage, and exclusion of allergic, parasitic, and malignant etiologies for hypereosinophilia [ 8 ]. Kikuchi disease, or histiocytic necrotizing lymphadenitis, is a rare etiology of lymphadenopathy [ 9 ]. It is a benign, self-limited disease presenting with fever, fatigue, and night sweats, sometimes accompanied by joint pain and a rash [ 9 ].…”

“…The differential for DRESS syndrome includes SJS, TEN, AGEP, lupus, lymphomas, hypereosinophilic syndromes, and Kikuchi disease [5][6][7][8][9]. DRESS syndrome, SJS, and TEN all represent forms of severe cutaneous drug-induced eruptions [12].…”

“…Kikuchi disease, or Fig. 5 The diffuse lymphadenopathy noted on CT abdomen/pelvis from admission histiocytic necrotizing lymphadenitis, is a rare etiology of lymphadenopathy [9]. It is a benign, self-limited disease presenting with fever, fatigue, and night sweats, sometimes accompanied by joint pain and a rash [9].…”

“…5 The diffuse lymphadenopathy noted on CT abdomen/pelvis from admission histiocytic necrotizing lymphadenitis, is a rare etiology of lymphadenopathy [9]. It is a benign, self-limited disease presenting with fever, fatigue, and night sweats, sometimes accompanied by joint pain and a rash [9]. Diagnosis requires analysis of clinical and histopathologic characteristics, subdivided into three types: early proliferative, necrotizing, and xanthomatous [9].…”

“…Differentials to consider alongside this condition include Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), acute generalized exanthematous pustulosis (AGEP), systemic lupus erythematosus, lymphomas, hypereosinophilic syndromes, and Kikuchi disease [5][6][7][8][9]. In this case, DRESS syndrome masqueraded as a lymphoproliferative disorder.…”