title: Co-occurrence of Kikuchi-Fujimoto's disease and Still's disease: case report and review of previously reported cases.
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.
authors.affiliation: Division of Rheumatology, Department of Medicine, New York University School of Medicine/NYU Langone Medical Center, New York, NY, USA.|Department of Rheumatology, NYU Hospital for Joint Diseases, Room 1410, 301 E 17th Street, New York, NY, 10003, USA.
authors.forename: Karen A
authors.initials: KA
authors.lastname: Toribio
authors.affiliation: Dermatopathology Section, New York University School of Medicine/NYU Langone Medical Center, New York, NY, USA.
authors.forename: Hideko
authors.initials: H
authors.lastname: Kamino
authors.affiliation: Ronald O. Perelman Department of Dermatology, New York University School of Medicine/NYU Langone Medical Center, New York, NY, USA.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Hu
authors.affiliation: Ronald O. Perelman Department of Dermatology, New York University School of Medicine/NYU Langone Medical Center, New York, NY, USA.
authors.forename: Miriam
authors.initials: M
authors.lastname: Pomeranz
authors.affiliation: Division of Rheumatology, Department of Medicine, New York University School of Medicine/NYU Langone Medical Center, New York, NY, USA. michael.pillinger@nyumc.org.|Department of Rheumatology, NYU Hospital for Joint Diseases, Room 1410, 301 E 17th Street, New York, NY, 10003, USA. michael.pillinger@nyumc.org.
authors.forename: Michael H
authors.initials: MH
authors.lastname: Pillinger
mesh_terms: D018501:Antirheumatic Agents / Q000627:therapeutic use; D005076:Exanthema / Q000209:etiology / Q000473:pathology; D005334:Fever / Q000209:etiology; D020042:Histiocytic Necrotizing Lymphadenitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D008206:Lymphatic Diseases / Q000209:etiology; D008297:Male; D016706:Still's Disease, Adult-Onset / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D055815:Young Adult
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein
keywords: Kikuchi; Kikuchi-Fujimoto’s disease; Overlap; Still’s disease
references.citation: Int J Hematol. 2003 May;77(4):359-63
references.pmid: 12774924
references.citation: Kansenshogaku Zasshi. 2009 Jul;83(4):363-8
references.pmid: 19697871
references.citation: Hum Pathol. 2010 Sep;41(9):1245-54
references.pmid: 20434191
references.citation: Am J Dermatopathol. 2011 Oct;33(7):736-9
references.pmid: 21915034
references.citation: Am J Pathol. 2001 Sep;159(3):915-24
references.pmid: 11549584
references.citation: N Engl J Med. 2012 Dec 20;367(25):2396-406
references.pmid: 23252526
references.citation: N Engl J Med. 2012 Dec 20;367(25):2385-95
references.pmid: 23252525
references.citation: Rev Rhum Engl Ed. 1997 May;64(5):352-3
references.pmid: 9190013
references.citation: Eur J Dermatol. 2012 Nov-Dec;22(6):798-9
references.pmid: 23113933
references.citation: Pathol Int. 2009 Sep;59(9):631-5
references.pmid: 19712130
references.citation: Rheumatol Int. 2012 Aug;32(8):2233-7
references.pmid: 22198666
references.citation: Int J Hematol. 2001 Jan;73(1):84-6
references.pmid: 11372760
references.citation: Br J Haematol. 1996 Dec;95(4):613-5
references.pmid: 8982035
references.citation: Mod Rheumatol. 2008;18(5):492-5
references.pmid: 18470474
references.citation: Appl Immunohistochem Mol Morphol. 2008 Mar;16(2):202-3
references.pmid: 18227719
references.citation: Proc Natl Acad Sci U S A. 1991 Oct 1;88(19):8485-9
references.pmid: 1924307
references.citation: Semin Arthritis Rheum. 2007 Dec;37(3):189-97
references.pmid: 17583775
references.citation: Int J Dermatol. 1999 Jun;38(6):464-7
references.pmid: 10397588
references.citation: Am J Clin Pathol. 2004 Jul;122(1):141-52
references.pmid: 15272543
references.citation: Rheumatol Int. 2012 May;32(5):1291-8
references.pmid: 21274538
references.citation: Ann Rheum Dis. 1995 Jul;54(7):587-90
references.pmid: 7668903
references.citation: J Rheumatol. 1988 Jun;15(6):981-3
references.pmid: 3418650
references.citation: Virchows Arch B Cell Pathol Incl Mol Pathol. 1992;62(4):263-9
references.pmid: 1359699
references.citation: J Rheumatol. 1992 Mar;19(3):424-30
references.pmid: 1578458
references.citation: J Rheumatol. 1990 Apr;17(4):568-9
references.pmid: 2348442