DOI: 10.12890/2025_006062

Adult-onset Still’s disease presenting with atypical Kikuchi-Fujimoto disease-like histiocytic necrotizing lymphadenitis: a diagnostic pitfall

Authors

  • Mykhailo Sosnov Department of Internal Medicine No. 2, Faculty of Medicine No. 2, Bogomolets National Medical University, Kyiv, Ukraine 0009-0006-5693-8104
  • Anna Shumeyko Department of Internal Medicine No. 2, Faculty of Medicine No. 2, Bogomolets National Medical University, Kyiv, Ukraine 0009-0002-0911-0241
  • Oleksandr Petrenko Department of General Surgery No. 1, Faculty of Medicine No. 2, Bogomolets National Medical University, Kyiv, Ukraine 0000-0002-7208-0412

msosnov97@gmail.com

Keywords

Adult-onset Still’s disease, Kikuchi-Fujimoto disease, hyperferritinaemia, autoinflammatory syndrome

Abstract

Background: Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disorder and a diagnosis of exclusion. Kikuchi-Fujimoto disease (KFD) is a benign, usually self-limiting necrotizing histiocytic lymphadenitis. The clinical and histopathological overlap between the two can be diagnostically challenging.
Case report: A 24-year-old female presented with a 6-week history of quotidian fever, migratory polyarthralgia, a fleeting salmon-coloured rash, and cervical lymphadenopathy. Initial workup revealed pronounced leucocytosis, highly elevated C-reactive protein, and critically high serum ferritin (6,500 µg/l). An excisional lymph node biopsy, performed to exclude malignancy, demonstrated features consistent with KFD. Active hemophagocytic lymphohistiocytosis (HLH) / macrophage activation syndrome (MAS) was ruled out as the calculated H-score was low (5), indicating a low probability of HLH, and the patient did not have the cytopenias and organomegaly necessary for a diagnosis of HLH/MAS. The patient’s presentation was ultimately confirmed as AOSD based on the Yamaguchi criteria.
Conclusion: This case illustrates a rare presentation where AOSD was masked by KFD-like lymphadenitis. Extreme hyperferritinaemia appears to serve as a vital, specific biomarker to establish the correct diagnosis of AOSD and ensure timely, life-saving immunomodulatory treatment. Also, prompt calculation of the H-score and the strict application of clinical and laboratory criteria are critical steps when managing patients with extreme hyperferritinaemia and systemic inflammation.

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References

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Published

2025-12-23

Issue

Vol. 13 No. 1 (2026)

Categories

How to Cite

1.
Sosnov M, Shumeyko A, Petrenko O. Adult-onset Still’s disease presenting with atypical Kikuchi-Fujimoto disease-like histiocytic necrotizing lymphadenitis: a diagnostic pitfall. EJCRIM [Internet]. 2025 Dec. 23 [cited 2026 Nov. 3];13(1). Available from: https://ejcrim.com/index.php/ejcrim/article/view/6062