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Co-occurrence of Kikuchi-Fujimoto’s disease and Still’s disease: case report and review of previously reported cases

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Abstract

Kikuchi-Fujimoto’s disease (KFD) and adult-onset Still’s disease (AOSD) are rare inflammatory conditions with some overlapping features. We encountered a 22-year-old male patient who presented with daily fevers, neck discomfort, and sore throat and subsequently developed rash, arthritis, and cervical lymphadenopathy. Biopsy of the skin rash was consistent with KFD skin involvement. Given that the patient also met criteria for AOSD, a final diagnosis of KFD/AOSD co-occurrence was made. Anti-IL-1β therapy with anakinra resulted in rapid resolution of all symptoms. A literature search identified eight more cases of KFD/AOSD. Fever, rash, arthritis, and lymphadenopathy were present in all patients. No case report demonstrated an association of rash eruption clearly associated with fever spikes. Duration of symptoms ranged from 3 weeks to 10 years. Seven patients had leukocytosis, six had anemia, and five demonstrated elevated ferritin and/or decreased glycosylated ferritin. Seven patients had elevated erythrocyte sedimentation rate (ESR), and seven had transaminitis. Eight of nine patients had no evidence of infectious disease. Autoantibodies were absent from all patients. KFD and AOSD are very rare diseases, yet they may overlap. The two conditions not only share several clinical and laboratory characteristics but also differ in characteristic ways. Given the rapid response observed with anakinra in the index patient, IL-1β likely plays a role in both diseases.

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References

  1. Kikuchi M (1972) Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes: a clinicopathological study. Acta Hematol Jpn 35:379–380

    Google Scholar 

  2. Fujimoto M, Kojima Y, Yamaguchi K (1972) Cervical subacute necrotizing lymphadenitis: a new clinicopathologic entity. Naika 20:920–927

    Google Scholar 

  3. Bosch X, Guilabert A, Miquel R, Campo E (2004) Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol 122(1):141–152

    Article  PubMed  Google Scholar 

  4. Cousin F, Grezard P, Roth B, Balme B, Gregoire-Bardel M, Perrot H (1999) Kikuchi disease associated with Still disease. Int J Dermatol 38(6):464–467

    Article  CAS  PubMed  Google Scholar 

  5. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19(3):424–430

    CAS  PubMed  Google Scholar 

  6. Kim HA, Sung JM, Suh CH (2012) Therapeutic responses and prognosis in adult-onset Still’s disease. Rheumatol Int 32(5):1291–1298

    Article  CAS  PubMed  Google Scholar 

  7. Kotter I, Wacker A, Koch S, Henes J, Richter C, Engel A, Gunaydin I, Kanz L (2007) Anakinra in patients with treatment-resistant adult-onset Still’s disease: four case reports with serial cytokine measurements and a review of the literature. Semin Arthritis Rheum 37(3):189–197

    Article  PubMed  Google Scholar 

  8. Ruperto N, Brunner HI, Quartier P, Constantin T, Wulffraat N, Horneff G, Brik R, McCann L, Kasapcopur O, Rutkowska-Sak L et al (2012) Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med 367(25):2396–2406

    Article  CAS  PubMed  Google Scholar 

  9. De Benedetti F, Brunner HI, Ruperto N, Kenwright A, Wright S, Calvo I, Cuttica R, Ravelli A, Schneider R, Woo P et al (2012) Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis. N Engl J Med 367(25):2385–2395

    Article  PubMed  Google Scholar 

  10. Ohta A, Matsumoto Y, Ohta T, Kaneoka H, Yamaguchi M (1988) Still’s disease associated with necrotizing lymphadenitis (Kikuchi's disease): report of 3 cases. J Rheumatol 15(6):981–983

    CAS  PubMed  Google Scholar 

  11. Lyberatos C (1990) Two more cases of Still's disease and Kikuchi's. J Rheumatol 17(4):568–569

    CAS  PubMed  Google Scholar 

  12. Miura T, Yamamoto T (2012) Adult-onset Still’s disease presenting lupus erythematosus-like facial erythema associated with Kikuchi's disease. Eur J Dermatol 22(6):798–799

    PubMed  Google Scholar 

  13. Garazzi S, Grossin M, Kahr MF (1997) Adult onset Still’s disease and Kikuchi’s disease. A new case. Rev Rhum Engl Educ 64(5):352–353

    CAS  Google Scholar 

  14. Yamamoto T (2012) Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values. Rheumatol Int 32(8):2233–2237

    Article  PubMed  Google Scholar 

  15. Kim JH, Kim YB, Il In S, Kim YC, Han JH (2010) The cutaneous lesions of Kikuchi’s disease: a comprehensive analysis of 16 cases based on the clinicopathologic, immunohistochemical, and immunofluorescence studies with an emphasis on the differential diagnosis. Hum Pathol 41(9):1245–1254

    Article  PubMed  Google Scholar 

  16. Pileri SA, Facchetti F, Ascani S, Sabattini E, Poggi S, Piccioli M, Rondelli D, Vergoni F, Zinzani PL, Piccaluga PP et al (2001) Myeloperoxidase expression by histiocytes in Kikuchi’s and Kikuchi-like lymphadenopathy. Am J Pathol 159(3):915–924

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  17. Nomura Y, Takeuchi M, Yoshida S, Sugita Y, Niino D, Kimura Y, Shimizu K, Aoki R, Suefuji N, Hirose S et al (2009) Phenotype for activated tissue macrophages in histiocytic necrotizing lymphadenitis. Pathol Int 59(9):631–635

    Article  CAS  PubMed  Google Scholar 

  18. Woods MT, Gavino AC, Burford HN, Hughey SL, Mercado P, Danila M, Andea AA (2011) The evolution of histopathologic findings in adult Still disease. Am J Dermatopathol 33(7):736–739

    Article  PubMed  Google Scholar 

  19. Nakamura I, Imamura A, Yanagisawa N, Suganuma A (2009) Ajisawa A: [Medical study of 69 cases diagnosed as Kikuchi’s disease]. Kansenshogaku Zasshi 83(4):363–368

    PubMed  Google Scholar 

  20. Magadur-Joly G, Billaud E, Barrier JH, Pennec YL, Masson C, Renou P, Prost A (1995) Epidemiology of adult Still's disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 54(7):587–590

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  21. Singh YP, Agarwal V, Krishnani N, Misra R (2008) Enthesitis-related arthritis in Kikuchi-Fujimoto disease. Mod Rheumatol Jpn Rheum Assoc 18(5):492–495

    Article  Google Scholar 

  22. Fernandez-Flores A, Bouso M, Alonso A, Manjon JA (2008) The histiocytic component of cutaneous manifestations of Kikuchi disease expresses myeloperoxidase. Appl Immunohistochem Mol Morphol 16(2):202–203

    Article  PubMed  Google Scholar 

  23. Sumiyoshi Y, Kikuchi M, Takeshita M, Yoneda S, Kobari S, Ohshima K (1992) Immunohistological study of skin involvement in Kikuchi’s disease. Virchows Arch B Cell Pathol Incl Mol Pathol 62(4):263–269

    Article  CAS  PubMed  Google Scholar 

  24. Kubota M, Tsukamoto R, Kurokawa K, Imai T, Furusho K (1996) Elevated serum interferon gamma and interleukin-6 in patients with necrotizing lymphadenitis (Kikuchi’s disease). Br J Haematol 95(4):613–615

    Article  CAS  PubMed  Google Scholar 

  25. Kato K, Ohshima K, Anzai K, Suzumiya J, Kikuchi M (2001) Elevated serum-soluble Fas ligand in histiocytic necrotizing lymphadenitis. Int J Hematol 73(1):84–86

    Article  CAS  PubMed  Google Scholar 

  26. Abe Y, Ohshima K, Nakashima M, Hara K, Matsushima T, Choi I, Nishimura J, Kikuchi M, Nawata H, Watanabe T et al (2003) Expression of apoptosis-associated protein RCAS1 in macrophages of histiocytic necrotizing lymphadenitis. Int J Hematol 77(4):359–363

    Article  CAS  PubMed  Google Scholar 

  27. Hogquist KA, Nett MA, Unanue ER, Chaplin DD (1991) Interleukin 1 is processed and released during apoptosis. Proc Natl Acad Sci U S A 88(19):8485–8489

    Article  PubMed Central  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, New York University School of Medicine/NYU Langone Medical Center, New York, NY, USA

    Karen A. Toribio & Michael H. Pillinger

  2. Dermatopathology Section, New York University School of Medicine/NYU Langone Medical Center, New York, NY, USA

    Hideko Kamino

  3. Ronald O. Perelman Department of Dermatology, New York University School of Medicine/NYU Langone Medical Center, New York, NY, USA

    Stephanie Hu & Miriam Pomeranz

  4. Department of Rheumatology, NYU Hospital for Joint Diseases, Room 1410, 301 E 17th Street, New York, NY, 10003, USA

    Karen A. Toribio & Michael H. Pillinger

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  1. Karen A. Toribio
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  2. Hideko Kamino
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  3. Stephanie Hu
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  4. Miriam Pomeranz
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  5. Michael H. Pillinger
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Correspondence to Michael H. Pillinger.

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Toribio, K.A., Kamino, H., Hu, S. et al. Co-occurrence of Kikuchi-Fujimoto’s disease and Still’s disease: case report and review of previously reported cases. Clin Rheumatol 34, 2147–2153 (2015). https://doi.org/10.1007/s10067-014-2755-3

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  • DOI: https://doi.org/10.1007/s10067-014-2755-3

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