Abstract
Kikuchi-Fujimoto’s disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.
Similar content being viewed by others
References
Kikuchi M (1972) Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. Acta Hematol Jpn 35:379–380
Fujimoto Y, Kojima Y, Yamaguchi K (1972) Cervical subacute necrotizing lymphadenitis. Naika 20:920–927
Dorfman RF, Berry GJ (1988) Kikuchi’s histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 5:329–345
Komócsi A, Tóvari E, Pajor L, Czirják L (2001) Histiocytic necrotizing lymphadenitis preceding systemic lupus erythematosus. J Eur Acad Dermatol Venereol 15:476–480
Graham LE (2002) Kikuchi-Fujimoto disease and peripheral arthritis: a first! Ann Rheum Dis 61:475
Litwin MD, Kirkham B, Henderson DR, Milazzo SC (1992) Histiocytic necrotising lymphadenitis in systemic lupus erythematosus. Ann Rheum Dis 51:805–807
El-Ramahi KM, Karrar A, Ali MA (1994) Kikuchi disease and its association with systemic lupus erythematosus. Lupus 3:409–411
Bousquet E, Tubéry M, Brousset P, Anzieu B, Dubarry B, Massip P, et al. (1996) Kikuchi syndrome, Hashimoto thyroiditis and lupus serology. Apropos of a case. Rev Med Interne 17:836–838
Sire S, Djossou F, Deminière C, Constans J, Ragnaud JM, Aubertin J (1996) Kikuchi-Fujimoto necrotizing histiocytic lymphadenitis: apropos of 2 cases and review of the literature. Rev Med Interne 17:842–845
Biasi D, Caramaschi P, Carletto A, Residori C, Randon M, Friso S, et al. (1996) Three clinical reports of Kikuchi’s lymphadenitis combined with systemic lupus erythematosus. Clin Rheumatol 15:81–83
Adoue D, Rauzy O, Rigal-Huguet F (1997) Kikuchi syndrome, cytomegalovirus infection and lupus. Rev Med Interne 18:338
Martínez-Vázquez C, Hughes G, Bordon J, Alonso-Alonso J, Anibarro-Garcia A, Redondo-Martínez E, et al. (1997) Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto’s disease, associated with systemic lupus erythematosus. QJM 90:531–533
Al Salloum AA (1998) Kikuchi’s disease and systemic lupus erythematosus in a Saudi child. Ann Saudi Med 18:51–53
Lopez C, Oliver M, Olavarria R, Sarabia MA, Chopite M (2000) Kikuchi-Fujimoto necrotizing lymphadenitis associated with cutaneous lupus erythematosus: a case report. Am J Dermatopathol 22:328–333
Dalkiliç E, Karakoç Y, Tolunay S, Yurtkuran M (2001) Systemic lupus erythematosus presenting as Kikuchi-Fujimoto disease. Clin Exp Rheumatol 19:226
Vilá LM, Mayor AM, Silvestrini IE (2001) Therapeutic response and long-term follow-up in a systemic lupus erythematosus patient presenting with Kikuchi’s disease. Lupus 10:126–128
Jiménez Sáenz JM, Llorente Arenas EM, Fuentes Solsona F, de Miguel García F, Alvarez Alegret R (2001) Kikuchi-Fujimoto’s disease and the association with systemic lupus erythematosus. An Med Interna 18:429–431
Ben Ghorbel I, Houman MH, Lamloum M, Khanfir M, Miled M, Kchir N, et al. (2002) Concomitant association of Kikuchi disease and systemic lupus erythematosus. Case report. Rev Med Interne 23:797–799
Quintás-Cardama A, Fraga M, Cozzi SN, Caparrini A, Maceiras F, Forteza J (2003) Fatal Kikuchi-Fujimoto disease: the lupus connection. Ann Hematol 82:186–188
Martins EP, Tanure LA, Sasaki M, Viana RS, Carvalho GT, Nicodemo EL, et al. (2003) Doença de Kikuchi-Fujimoto. Relato de três casos e revisão de literatura. Rev Bras Reumatol 43:62–68
Danowski A, Bica B, Baptista R, Nentzinsky W, Azavedo MN (2003) Linfadenite de Kikuchi associada ao lúpus eritematoso sistêmico. Rev Bras Reumatol 43:58–61
Hu S, Kuo TT, Hong HS (2003) Lupus lymphadenitis simulating Kikuchi’s lymphadenitis in patients with systemic lupus erythematosus: a clinicopathological analysis of six cases and review of the literature. Pathol Int 53:221–226
Wano Y, Ebata K, Masaki Y, Takeshita S, Ogawa N, Kim CG, et al. (2000) Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto’s disease) accompanied by hemophagocytosis and salivary gland swelling in a patient with systemic lupus erythematosus. Rinsho Ketsueki 41:54–60
Tumiati B, Bellelli A, Portioli I, Prandi S (1991) Kikuchi’s disease in systemic lupus erythematosus: an independent or dependent event? Clin Rheumatol 10:90–93
Meyer O, Ribard P, Belmatoug N, Kahn MF, Grossin M, Fournet JC, et al. (1991) 3 cases of Kituchi’s lymphadenitis in systemic lupus erythematosus. Role of the parvovirus B19. Ann Med Interne (Paris) 142:259–264
Raison-Peyron N, Meunier L, Diebold J, Meynadier J (1996) Kikuchi’s disease and systemic lupus erythematosus. Eur J Dermatol 6:447–449
Menasce LP, Banerjee SS, Edmondson D, Harris M (1998) Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease): continuing diagnostic difficulties. Histopathology 33:248–254
Norris AH, Krasinskas AM, Salhany KE, Gluckman SJ (1996) Kikuchi-Fujimoto disease: a benign cause of fever and lymphadenopathy. Am J Med 101:401–405
Spies J, Foucar K, Thompson CT, LeBoit PE (1999) The histopathology of cutaneous lesions of Kikuchi’s disease (necrotizing lymphadenitis): a report of five cases. Am J Surg Pathol 23:1040–1047
Kaur S, Thami GP, Kanwar AJ (2002) Kikuchi’s disease, skin and systemic lupus erythematosus. Br J Dermatol 146:167–168
Okuzawa C, Kuroiwa T, Kaneko Y, Ueki K, Tsukada Y, Nojima Y (2003) Kikuchi’s disease accompanied by lupus-like butterfly rash. J Rheumatol 30:857–859
Yasukawa K, Matsumura T, Sato-Matsumura KC, Takahashi T, Fujioka Y, Kobayashi H, et al. (2001) Kikuchi’s disease and the skin: case report and review of the literature. Br J Dermatol 144:885–889
Iguchi H, Sunami K, Yamane H, Konishi K, Takayama M, Nakai Y, et al. (1998) Apoptotic cell death in Kikuchi’s disease: a TEM study. Acta Otolaryngol 53 [Suppl 8]:250–253
Acknowledgement
We thank Dr. Almir Ramos Junior for helping with the histopathological analysis.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Santana, A., Lessa, B., Galrão, L. et al. Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature. Clin Rheumatol 24, 60–63 (2005). https://doi.org/10.1007/s10067-004-0923-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-004-0923-6