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Kikuchi Fujimoto disease with systemic lupus erythematosus: a rare association
Indian Journal of Rheumatology 2006 June Volume 1, Number 1; pp. 36–37
Case Report
Kikuchi Fujimoto disease with systemic lupus erythematosus: a rare association R Saigal, RN Yadav, S Shrivastava, V Mathur, V Mittal, M Mittal
ABSTRACT Kikuchi Fujimoto disease is a rare disease which must be considered as one of the causes of prolonged fever of unknown cause and lymphadenopathy. Rarely it may antedate, postdate or coincide with the diagnosis of systemic lupus erythematosus. Present case, is an example of this association.
Keywords: Kikuchi Fujimoto disease, lymphadenopathy, systemic lupus erythematosus.
CASE REPORT A 23-year-old female presented with low grade fever and polyarthralgia for last 2 months. There was history of photosensitivity, recurrent oral ulcer and alopecia for the last 1 year. There was no history of parotid enlargement, sicca symptoms, proximal muscle weakness, Raynaud’s phenomenon or dysphagia. Examination revealed low grade fever, moderate anaemia and cervical lymph node enlargement (2–3 cm, mobile, tender, firm, not matted, and 4 or 5 in number in the posterior triangle). Joints were tender but without swelling. Erythematous malar rash was present. On investigation, following results were obtained: Hb = 7.8 g/dl, TLC = 3025 cells/mm3, ESR = 130 mm in first hour, MCV = 75.3 fl, MCH = 23.5 pg/cell, platelet count = 1.5 lakh/mm3, peripheral blood film for malaria parasite was negative, urine examination was normal, blood and urine cultures were sterile. Blood glucose, renal and liver function tests were within normal range. x-Ray of chest and hands were normal. C-reactive protein (CRP), rheumatoid factors (RF) and antinuclear antibody (ANA) by indirect immunofluorescence (IIF) were positive in significant titre. Antibody to Sjögren’s syndrome A (SSA; Ro) was positive while antibody to Sjögren’s syndrome B (SSB; La) was negative. Lymph node biopsy (Figs 1 and 2) showed necrosis in para-cortical area with nuclear debris and infiltration by mononuclear cells. The histological picture was consistent with Kikuchi Fujimoto disease (KFD). The absence of neutrophils and plasma cells favour this Department of Medicine, SMS Medical College, Jaipur, Rajasthan, India. Correspondence: Dr. Renu Saigal, email: [email protected]
diagnosis as against that of lupus-associated necrotising lymphadenitis.
DISCUSSION Kikuchi Fujimoto disease is extremely uncommon, benign and self-limited. It is known to have worldwide distribution with higher prevalence among Japanese and Asians.1 The disease is more common in females < 30 years of age. Aetiology of KFD is not known, however, viral or autoimmune factors have been suggested. KFD patients present with low-grade fever associated with upper respiratory tract symptoms and tender cervical node enlargement in posterior triangle of neck as in the cited case. Diagnosis is established by excisional biopsy showing characteristic changes1 (Figs 1 and 2). RF and ANA are usually negative in patients with KFD but association between KFD and SLE has been reported with a frequency probably greater than that expected by chance alone. KFD may antedate, postdate or coincide with the diagnosis of SLE1, 2 as in the present case. Six cases of pre-SLE or post-SLE necrotising lymphadenitis were found to be true KFD.3 Thus a patient with KFD should be assessed for SLE and should have a longterm follow-up, checking for development of SLE. KFD should be ruled out in SLE flare with lymphadenitis.3 KFD is generally self-limited and lasts for 1–4 months. Recurrence rate is only 3–4%.4 So far, only three cases of
Kikuchi Fujimoto disease with SLE
.ECKHA Lymph node biopsy showing paracortical areas of coagulative necrosis (arrow) (H&E stained; 10x).
fatal KFD are reported. Treatment is on symptomatic lines. Nonsteroidal anti-inflammatory drugs (NSAIDs), antipyretics and low-dose corticosteroids may be given (if NSAID fails). SLE, if associated, has to be treated according to standard guidelines.
Case Report
.ECKHA Lymph node biopsy showing large number of histiocytes (arrow) and nuclear debris (arrowhead) (H&E stained; 40x).
2.
3.
REFERENCES 1.
Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol 2004; 122: 141–52.
37
4.
Martinez-Vazquez C, Hughes G, Bordon J, Alonso-Alonso J, Anibarro-Garcia A, Redondo-Martinez E, et al. Histocytic necrotizing lymphadenitis, Kikuchi. Fujimoto’s disease, associated with systemic lupus erythematosus. QJM 1997; 90: 531–3. Hu S, Kuo TT, Hong HS. 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 2003; 53: 221–6. Dorfman RF. Histocytic necrotizing lymphadenitis of Kikuchi and Fujimoto. Arch Pathol Lab Med 1987; 111: 1026–9.
Case Report
Kikuchi Fujimoto disease with systemic lupus erythematosus: a rare association R Saigal, RN Yadav, S Shrivastava, V Mathur, V Mittal, M Mittal
ABSTRACT Kikuchi Fujimoto disease is a rare disease which must be considered as one of the causes of prolonged fever of unknown cause and lymphadenopathy. Rarely it may antedate, postdate or coincide with the diagnosis of systemic lupus erythematosus. Present case, is an example of this association.
Keywords: Kikuchi Fujimoto disease, lymphadenopathy, systemic lupus erythematosus.
CASE REPORT A 23-year-old female presented with low grade fever and polyarthralgia for last 2 months. There was history of photosensitivity, recurrent oral ulcer and alopecia for the last 1 year. There was no history of parotid enlargement, sicca symptoms, proximal muscle weakness, Raynaud’s phenomenon or dysphagia. Examination revealed low grade fever, moderate anaemia and cervical lymph node enlargement (2–3 cm, mobile, tender, firm, not matted, and 4 or 5 in number in the posterior triangle). Joints were tender but without swelling. Erythematous malar rash was present. On investigation, following results were obtained: Hb = 7.8 g/dl, TLC = 3025 cells/mm3, ESR = 130 mm in first hour, MCV = 75.3 fl, MCH = 23.5 pg/cell, platelet count = 1.5 lakh/mm3, peripheral blood film for malaria parasite was negative, urine examination was normal, blood and urine cultures were sterile. Blood glucose, renal and liver function tests were within normal range. x-Ray of chest and hands were normal. C-reactive protein (CRP), rheumatoid factors (RF) and antinuclear antibody (ANA) by indirect immunofluorescence (IIF) were positive in significant titre. Antibody to Sjögren’s syndrome A (SSA; Ro) was positive while antibody to Sjögren’s syndrome B (SSB; La) was negative. Lymph node biopsy (Figs 1 and 2) showed necrosis in para-cortical area with nuclear debris and infiltration by mononuclear cells. The histological picture was consistent with Kikuchi Fujimoto disease (KFD). The absence of neutrophils and plasma cells favour this Department of Medicine, SMS Medical College, Jaipur, Rajasthan, India. Correspondence: Dr. Renu Saigal, email: [email protected]
diagnosis as against that of lupus-associated necrotising lymphadenitis.
DISCUSSION Kikuchi Fujimoto disease is extremely uncommon, benign and self-limited. It is known to have worldwide distribution with higher prevalence among Japanese and Asians.1 The disease is more common in females < 30 years of age. Aetiology of KFD is not known, however, viral or autoimmune factors have been suggested. KFD patients present with low-grade fever associated with upper respiratory tract symptoms and tender cervical node enlargement in posterior triangle of neck as in the cited case. Diagnosis is established by excisional biopsy showing characteristic changes1 (Figs 1 and 2). RF and ANA are usually negative in patients with KFD but association between KFD and SLE has been reported with a frequency probably greater than that expected by chance alone. KFD may antedate, postdate or coincide with the diagnosis of SLE1, 2 as in the present case. Six cases of pre-SLE or post-SLE necrotising lymphadenitis were found to be true KFD.3 Thus a patient with KFD should be assessed for SLE and should have a longterm follow-up, checking for development of SLE. KFD should be ruled out in SLE flare with lymphadenitis.3 KFD is generally self-limited and lasts for 1–4 months. Recurrence rate is only 3–4%.4 So far, only three cases of
Kikuchi Fujimoto disease with SLE
.ECKHA Lymph node biopsy showing paracortical areas of coagulative necrosis (arrow) (H&E stained; 10x).
fatal KFD are reported. Treatment is on symptomatic lines. Nonsteroidal anti-inflammatory drugs (NSAIDs), antipyretics and low-dose corticosteroids may be given (if NSAID fails). SLE, if associated, has to be treated according to standard guidelines.
Case Report
.ECKHA Lymph node biopsy showing large number of histiocytes (arrow) and nuclear debris (arrowhead) (H&E stained; 40x).
2.
3.
REFERENCES 1.
Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol 2004; 122: 141–52.
37
4.
Martinez-Vazquez C, Hughes G, Bordon J, Alonso-Alonso J, Anibarro-Garcia A, Redondo-Martinez E, et al. Histocytic necrotizing lymphadenitis, Kikuchi. Fujimoto’s disease, associated with systemic lupus erythematosus. QJM 1997; 90: 531–3. Hu S, Kuo TT, Hong HS. 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 2003; 53: 221–6. Dorfman RF. Histocytic necrotizing lymphadenitis of Kikuchi and Fujimoto. Arch Pathol Lab Med 1987; 111: 1026–9.