eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Cutaneous Kikuchi Disease: Differential Diagnoses & Workup

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, New York Medical College-Metropolitan Hospital; Private Practice
Contributor Information and Disclosures

Updated: Feb 12, 2008

Differential Diagnoses

Atypical Mycobacterial Diseases

Other Problems to Be Considered

Lymphoma
Leukemia
Systemic lupus erythematosus
Sweet syndrome
Hodgkin disease

Workup

Laboratory Studies

  • Patients with KD can have elevated erythrocyte sedimentation rates, neutropenia, and atypical lymphocytosis.
  • Some reports note that leukopenia occurs in 25-43% of patients; leukocytosis, in 2.9-5% of patients; and atypical lymphocytosis, in 25% of patients.
  • Murine and in vitro experiments have shown that the expression/induction of T-bet, the master regulator of Th1 differentiation, can be achieved by obligate intracellular pathogens involved with lymphadenitis and by KD, suggesting the KD is somehow related to obligate intracellular pathogens.18
  • Eboriadou et al19 reported KD associated with extreme leukopenia and thrombocytopenia in an adolescent boy.

Imaging Studies

  • Many different patterns of computed tomographic appearance of KD have been reported. They include enlarged lymph nodes with a hypodense center and peripheral ring enhancement.
  • Many small, clustered lymph nodes may be a characteristic imaging feature of KD.
  • The abdominal extent of disease may be underreported if cross-sectional imaging is not performed.
  • Kim et al20 described a 10-year-old girl with KD that mimicked malignant lymphoma on fluorodeoxyglucose positron emission tomography scans.

Other Tests

  • Flow cytometric studies performed on the affected lymph node, bone marrow aspirate, and peripheral blood reveal a relative expansion of mature activated T lymphocytes, predominantly expressing a CD8+ phenotype.

Procedures

  • Lymph node biopsy and skin biopsy are useful procedures.

Histologic Findings

Skin biopsy specimens show a dense, lymphohistiocytic, superficial, deep perivascular and interstitial infiltrate; papillary dermal edema; and abundant nuclear debris with a conspicuous absence of neutrophils, paralleling the nodal histology of KD.

CD68 immunohistochemistry shows many plasmacytoid monocytes. CD3, CD4, and CD8 immunohistochemistry shows highly variable staining. Only rare staining with T-cell intracytoplasmic antigen and CD30 occurs.

With immunofluorescence, deposition of immunoglobulins and complement can be seen at the dermoepidermal junction and in the walls of dermal blood vessels.

In one case, histologic findings from a cutaneous lesion revealed dermal patchy infiltrates composed of large lymphoreticular cells and scattered cells that resembled Hodgkin or Reed-Sternberg cells. Subsequent immunohistochemical studies demonstrated they were activated fibroblasts.

In 2004, Yen et al21 suggested that interface change might be one of the pathological features of the cutaneous manifestations of Kikuchi-Fujimoto disease.

More on Cutaneous Kikuchi Disease

Overview: Cutaneous Kikuchi Disease
Differential Diagnoses & Workup: Cutaneous Kikuchi Disease
Treatment & Medication: Cutaneous Kikuchi Disease
Follow-up: Cutaneous Kikuchi Disease
References
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References

  1. Zhang WP, Wang JH, Wang WQ, Chen XQ, Wang Z, Li YF, et al. An association between parvovirus B19 and Kikuchi-Fujimoto disease. Viral Immunol. Sep 2007;20(3):421-8. [Medline].

  2. Chuang CH, Yan DC, Chiu CH, Huang YC, Lin PY, Chen CJ, et al. Clinical and laboratory manifestations of Kikuchi's disease in children and differences between patients with and without prolonged fever. Pediatr Infect Dis J. Jun 2005;24(6):551-4. [Medline].

  3. Rau AR, Kini H. Clinicopathological study of Kikuchi's disease. Indian J Pathol Microbiol. Jul 2007;50(3):485-8. [Medline].

  4. Lima M, Silvestre F, Correia J, Oliveira J, Justiça B. Kikuchi's disease: a case report with emphasis on flow cytometric studies. Sangre (Barc). Oct 1996;41(5):383-6. [Medline].

  5. Harris VK, Danda D, Murali NS, Das PK, Abraham M, Cherian AM, et al. Unusual association of Kikuchi's disease and dengue virus infection evolving into systemic lupus erythematosus. J Indian Med Assoc. Jul 2000;98(7):391-3. [Medline].

  6. Viseux V, Schoenlaub P, Marciniak S, Staroz F, Plantin P. [Kikuchi's disease associated with cutaneous manifestations evoking Well's syndrome]. Ann Dermatol Venereol. Dec 2003;130(12 Pt 1):1150-3. [Medline].

  7. Join-Lambert OF, Ouache M, Canioni D, Beretti JL, Blanche S, Berche P, et al. Corynebacterium pseudotuberculosis necrotizing lymphadenitis in a twelve-year-old patient. Pediatr Infect Dis J. Sep 2006;25(9):848-51. [Medline].

  8. Carlson JA, Perlmutter A, Tobin E, Richardson D, Rohwedder A. Adverse antibiotic-induced eruptions associated with epstein barr virus infection and showing Kikuchi-Fujimoto disease-like histology. Am J Dermatopathol. Feb 2006;28(1):48-55. [Medline].

  9. Kim KJ, Jee MS, Chang SE, Choi JH, Sung KJ, Moon KC, et al. Kikuchi-Fujimoto disease with papulopustular skin manifestations. Clin Exp Dermatol. Mar 2003;28(2):142-4. [Medline].

  10. Imai K, Yokozeki H, Nishioka K. Kikuchi's disease (histiocytic necrotizing lymphadenitis) with cutaneous involvement. J Dermatol. Sep 2002;29(9):587-92. [Medline].

  11. Kaur S, Thami GP, Mohan H, Kanwar AJ. Kikuchi disease with facial rash and erythema multiforme. Pediatr Dermatol. Sep-Oct 2001;18(5):403-5. [Medline].

  12. Rao GS, Vohra D, Kuruvilla M. Is Kikuchi-Fujimoto disease a manifestation of systemic lupus erythematosus?. Int J Dermatol. Apr 2006;45(4):454-6. [Medline].

  13. Roger M, Hopfner C, Loiselet G, Libbrecht E, Bressieux JM, Fur A. [Eyelid edema revealing Kikuchi's disease]. Ann Dermatol Venereol. Nov 1999;126(11):826-8. [Medline].

  14. Letawe C, Pierard-Franchimont C, Rustom KA, et al. [Cutaneous-lymph node Kikuchi-Fujimoto disease]. Ann Dermatol Venereol. Jan 1999;126(1):26-8. [Medline].

  15. Kim HS, Oh ST. Kikuchi disease with transient eruption in a 7-year-old boy. Pediatr Dermatol. Jul-Aug 2007;24(4):459-60. [Medline].

  16. Huh J, Chi HS, Kim SS, Gong G. A study of the viral etiology of histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease). J Korean Med Sci. Feb 1998;13(1):27-30. [Medline].

  17. Aqel N, Henry K, Woodrow D. Skin involvement in Kikuchi's disease: an immunocytochemical and immunofluorescence study. Virchows Arch. Apr 1997;430(4):349-52. [Medline].

  18. Jöhrens K, Anagnostopoulos I, Dürkop H, Stein H. Different T-bet expression patterns characterize particular reactive lymphoid tissue lesions. Histopathology. Mar 2006;48(4):343-52. [Medline].

  19. Eboriadou M, Tragiannidis A, Markou K, Athanassiadou F. Kikuchi-fujimoto disease associated with extreme leukopenia and thrombocytopenia in an adolescent boy. J Pediatr Hematol Oncol. Dec 2007;29(12):864. [Medline].

  20. Kim CH, Hyun O J, Yoo IeR, Kim SH, Sohn HS, Chung SK. Kikuchi Disease Mimicking Malignant Lymphoma on FDG PET/CT. Clin Nucl Med. Sep 2007;32(9):711-2. [Medline].

  21. Yen HR, Lin PY, Chuang WY, Chang ML, Chiu CH. Skin manifestations of Kikuchi-Fujimoto disease: case report and review. Eur J Pediatr. Apr 2004;163(4-5):210-3. [Medline].

  22. Khan FY, Morad NA, Fawzy Z. Kikuchi's disease associated with hemophagocytosis. Chang Gung Med J. Jul-Aug 2007;30(4):370-3. [Medline].

  23. Bachi R. Kikuchi disease in a connective tissue disorder. Med J Malaysia. Sep 2002;57(3):357-60. [Medline].

  24. Goteri G, Filosa A, Pieramici T, Fioravanti P, Barocci G, Bearzi I. Cutaneous Kikuchi's disease: report of a new case and review of the literature. Histopathology. Sep 2004;45(3):300-1. [Medline].

  25. Kumaran MS, Dogra S, Saikia UN, Kanwar AJ. Kikuchi's disease with skin lesions in a patient with SLE. J Eur Acad Dermatol Venereol. Nov 2005;19(6):783-4. [Medline].

  26. Kuo TT. Cutaneous manifestation of Kikuchi's histiocytic necrotizing lymphadenitis. Am J Surg Pathol. Sep 1990;14(9):872-6. [Medline].

  27. Lopez C, Oliver M, Olavarria R, et al. Kikuchi-Fujimoto necrotizing lymphadenitis associated with cutaneous lupus erythematosus: a case report. Am J Dermatopathol. Aug 2000;22(4):328-33. [Medline].

  28. Spies J, Foucar K, Thompson CT, LeBoit PE. The histopathology of cutaneous lesions of Kikuchi's disease (necrotizing lymphadenitis): a report of five cases. Am J Surg Pathol. Sep 1999;23(9):1040-7. [Medline].

  29. Toll A, Gilaberte M, Matias-Guiu X, et al. Kikuchi's disease (necrotizing lymphadenitis) with cutaneous involvement associated with subacute cutaneous lupus erythematosus. Clin Exp Dermatol. May 2004;29(3):240-3. [Medline].

  30. Yasukawa K, Matsumura T, Sato-Matsumura KC, et al. Kikuchi''s disease and the skin: case report and review of the literature. Br J Dermatol. Apr 2001;144(4):885-9. [Medline].

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Further Reading

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Keywords

histiocytic necrotizing lymphadenitis, HNL, Kikuchi-Fujimoto disease, KD, Kikuchi's histiocytic necrotizing lymphadenitis, necrotizing lymphadenitis, lymphadenitis showing focal reticulum cell hyperplasia with  nuclear debris and phagocytosis, cervical subacute necrotizing lymphadenitis, Kikuchi's disease

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Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, New York Medical College-Metropolitan Hospital; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Shyam Verma, MBBS, DVD, FAAD, Adjunct Clinical Assistant Professor, Department of Dermatology, University of Virginia, State University of New York at Stonybrook, Penn State University
Shyam Verma, MBBS, DVD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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