Cutaneous Kikuchi Disease Clinical Presentation: History, Physical Examination, Complications

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Presentation

History

Kikuchi disease (KD) commonly presents with painful cervical lymphadenopathy accompanied by systemic symptoms such as fever, arthralgias, myalgias, anorexia, weight loss, night sweats, and chills. Hepatosplenomegaly and aseptic meningitis have also been reported.^{[3, 9]}Cutaneous lesions, known as cutaneous KD, are relatively common and may occur in about one third of patients.^[3]These cutaneous eruptions are varied and may include erythematous indurated papules or plaques, nodules, acneiform eruptions, or urticarial lesions. The eruption favors the upper body, especially the head and neck.^{[9, 14]}

A prior history of autoimmune disease is often elicited in patients with KD. One study reported that 12% of patients with KD had a preceding diagnosis of systemic lupus erythematosus, which is the most commonly associated autoimmune disease.^[3]Cutaneous lupus erythematosus, including discoid lupus and subacute cutaneous lupus, may also be associated with KD.^{[8, 9, 10, 11, 12, 22]}Other associated autoimmune diseases include systemic sclerosis, antiphospholipid antibody syndrome, rheumatoid arthritis, Hashimoto thyroiditis, and Wegner granulomatosis.^{[3, 23]}Therefore, it is important to take a thorough medical history in patients with possible KD to elicit any of these associated and possibly predisposing conditions.

Physical Examination

Physical examination most commonly demonstrates cervical lymphadenopathy in Kikuchi disease (KD), although other lymph nodes may be involved, such as the axillary or supraclavicular nodes. Lymphadenopathy is usually painful.^[9]Cutaneous KD occurs in about one third of patients (16-40%) and is often of a nonspecific nature.^{[3, 24]}Most cutaneous lesions present as erythematous macules, papules, plaques, nodules, or ulcers on the upper part of the body, such as the trunk, the upper extremities, and the face.^{[9, 14, 25]}

Several reports in the literature highlight the different physical examination findings associated with cutaneous KD. For example, Kim et al^[26]reported a case of KD with a papulopustular eruption over the torso, arms, and legs. Imai et al^[27]described a patient with lip edema. This patient had facial, neck, and arm desquamation and erosions, as well as transient indurated erythematous papules. Kaur et al^[28]described a patient with Kikuchi disease with erythema multiforme–like lesions and a facial rash.

Other atypical presentations include oral ulcers, vesicles,^[29]and eyelid edema.^[30]

Complications

Kikuchi disease (KD) usually resolves on its own in a matter of months without sequelae. However, KD can evolve into lupus erythematosus (either systemic or cutaneous). Therefore, long-term follow up and counseling patients about this risk is prudent.

Differential Diagnoses

Kikuchi M. Lymphadenitis showing focal reticulum cervical hyperplasia with nuclear debris and phagocytosis. Acta Hematol Jpn. 1972. 35:379-380.
Fujimoto Y, Kozima Y, Yamaguchi K. Cervical subacute necrotizing lymphadenitis: a new clinicopathologic entity. Naika. 1972. 20:920-27.
Dumas G, Prendki V, Haroche J, et al. Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature. Medicine (Baltimore). 2014 Nov. 93 (24):372-82. [Medline].
Dorfman RF, Berry GJ. Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol. 1988 Nov. 5 (4):329-45. [Medline].
5. Kim KH, Jung SH, Park C, Choi IJ. Subacute necrotizing lymphadenitis-a collective clinicopathological and immunohistochemical study. Yonsei Med J. 1992; 33:32–40. [Medline].
Lin HC, Su CY, Huang CC, Hwang CF, Chien CY. Kikuchi's disease: a review and analysis of 61 cases. Otolaryngol Head Neck Surg. 2003 May. 128 (5):650-3. [Medline].
Mathew LM, Kapila R, Schwartz RA. Kikuchi-Fujimoto disease: a diagnostic dilemma. Int J Dermatol. 2016 Oct. 55 (10):1069-75. [Medline].
Ruiz Beguerie J, Fernandez Penas P, Sharma R. Kikuchi-Fujimoto disease with cutaneous presentation in a patient with subacute cutaneous lupus erythematosus. Dermatol Online J. 2012 Sep 15. 18 (9):8. [Medline].
Perry AM, Choi SM. Kikuchi-Fujimoto Disease: A Review. Arch Pathol Lab Med. 2018 Nov. 142 (11):1341-1346. [Medline].
Lopez C, Oliver M, Olavarria R, Sarabia MA, Chopite M. Kikuchi-Fujimoto necrotizing lymphadenitis associated with cutaneous lupus erythematosus: a case report. Am J Dermatopathol. 2000 Aug. 22 (4):328-33. [Medline].
Lecoules S, Michel M, Zarrouk V, Gaulard P, Schaeffer A, Godeau B. [Recurrent Kikuchi's disease in a patient with discoid lupus]. Rev Med Interne. 2003 Sep. 24 (9):613-6. [Medline].
Silver SG, Hong HC, Ting PT, Ball NJ. Kikuchi-Fujimoto's necrotizing lymphadenitis in association with discoid lupus erthematosus: a case report. J Cutan Med Surg. 2004 Nov-Dec. 8 (6):442-5. [Medline].
Paradela S, Lorenzo J, Martínez-Gómez W, Yebra-Pimentel T, Valbuena L, Fonseca E. Interface dermatitis in skin lesions of Kikuchi-Fujimoto's disease: a histopathological marker of evolution into systemic lupus erythematosus?. Lupus. 2008 Dec. 17 (12):1127-35. [Medline].
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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. 1998 Feb. 13 (1):27-30. [Medline].
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Moinet F, Molinié V, Béraud G, Polomat K, Cordel N, Sainte-Marie D, et al. Epidemiology and Characteristics of Kikuchi-Fujimoto Disease in the African-Descent Population of Martinique, French West Indies. Arthritis Care Res (Hoboken). 2016 Dec. 68 (12):1883-1887. [Medline].
Kuo TT. Cutaneous manifestation of Kikuchi's histiocytic necrotizing lymphadenitis. Am J Surg Pathol. 1990 Sep. 14 (9):872-6. [Medline].
Jung IY, Ann HW, Kim JJ, Lee SJ, Kim J, Seong H, et al. The incidence and clinical characteristics by gender differences in patients with Kikuchi-Fujimoto disease. Medicine (Baltimore). 2017 Mar. 96 (11):e6332. [Medline].
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. 2005 Jun. 24 (6):551-4. [Medline].
Di Lernia V, Bajocchi G, Piana S. Subacute cutaneous lupus erythematosus onset preceded by Kikuchi-Fujimoto disease. Dermatol Pract Concept. 2014 Jan. 4 (1):47-9. [Medline].
DeFilipp Z. Cutaneous manifestation of Kikuchi-Fujimoto disease in the setting of granulomatosis with polyangiitis (Wegener's). J Gen Intern Med. 2012 Sep. 27 (9):1220-2. [Medline].
Atwater AR, Longley BJ, Aughenbaugh WD. Kikuchi's disease: case report and systematic review of cutaneous and histopathologic presentations. J Am Acad Dermatol. 2008 Jul. 59 (1):130-6. [Medline].
Letawe C, Piérard-Franchimont C, Rustom KA, Hermanns-Lê T, Piérard GE. [Cutaneous-lymph node Kikuchi-Fujimoto disease]. Ann Dermatol Venereol. 1999 Jan. 126 (1):26-8. [Medline].
Kim KJ, Jee MS, Chang SE, Choi JH, Sung KJ, Moon KC, et al. Kikuchi-Fujimoto disease with papulopustular skin manifestations. Clin Exp Dermatol. 2003 Mar. 28 (2):142-4. [Medline].
Imai K, Yokozeki H, Nishioka K. Kikuchi's disease (histiocytic necrotizing lymphadenitis) with cutaneous involvement. J Dermatol. 2002 Sep. 29 (9):587-92. [Medline].
Kaur S, Thami GP, Mohan H, Kanwar AJ. Kikuchi disease with facial rash and erythema multiforme. Pediatr Dermatol. 2001 Sep-Oct. 18 (5):403-5. [Medline].
Rao GS, Vohra D, Kuruvilla M. Is Kikuchi-Fujimoto disease a manifestation of systemic lupus erythematosus?. Int J Dermatol. 2006 Apr. 45 (4):454-6. [Medline].
Roger M, Hopfner C, Loiselet G, Libbrecht E, Bressieux JM, Fur A. [Eyelid edema revealing Kikuchi's disease]. Ann Dermatol Venereol. 1999 Nov. 126 (11):826-8. [Medline].
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. 2007 Dec. 29 (12):864. [Medline].
Shim EJ, Lee KM, Kim EJ, Kim HG, Jang JH. CT pattern analysis of necrotizing and nonnecrotizing lymph nodes in Kikuchi disease. PLoS One. 2017. 12 (7):e0181169. [Medline].
Lima M, Silvestre F, Correia J, Oliveira J, Justiça B. Kikuchi's disease: a case report with emphasis on flow cytometric studies. Sangre (Barc). 1996 Oct. 41 (5):383-6. [Medline].
Kim JH, Kim YB, In SI, Kim YC, Han JH. 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. 2010 Sep. 41 (9):1245-54. [Medline].
Khan FY, Morad NA, Fawzy Z. Kikuchi's disease associated with hemophagocytosis. Chang Gung Med J. 2007 Jul-Aug. 30 (4):370-3. [Medline].
Chen PH, Huang YF, Tang CW, Wann SR, Chang HT. Kikuchi-Fujimoto disease: an amazing response to hydroxychloroquine. Eur J Pediatr. 2010 Dec. 169 (12):1557-9. [Medline].
Honda F, Tsuboi H, Toko H, Ohyama A, Takahashi H, Abe S, et al. Recurrent Kikuchi-Fujimoto Disease Successfully Treated by the Concomitant Use of Hydroxychloroquine and Corticosteroids. Intern Med. 2017 Dec 15. 56 (24):3373-3377. [Medline].
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Media Gallery

Very high magnification micrograph of histiocytic necrotizing lymphadenitis, also known as Kikuchi disease. Hematoxylin and eosin stain. Courtesy of Nephron (own work), via Wikimedia Commons.

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Author

Jenny E Liles, MD Resident Physician, Department of Dermatology, Medical College of Georgia at Augusta University

Disclosure: Nothing to disclose.

Coauthor(s)

Loretta S Davis, MD Professor and Chair, Department of Dermatology, Medical College of Georgia, Augusta University

Loretta S Davis, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Association of Military Dermatologists, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Shyam Verma, MBBS, DVD, FAAD Clinical Associate Professor, Department of Dermatology, University of Virginia School of Medicine; Adjunct Associate Professor, Department of Dermatology, State University of New York at Stonybrook School of Medicine; Adjunct Associate Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Shyam Verma, MBBS, DVD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Noah S Scheinfeld, JD, MD, FAAD † Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Cutaneous Kikuchi Disease Clinical Presentation

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