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Kikuchi-Fujimoto disease is a subacute necrotising lymphadenopathy of unknown cause. It is more common in young Asian women, usually affects cervical lymph nodes, and is characterised histologically by histiocytic proliferation and necrosis of lymph nodes. Clinically the lymphadenopathy is painless, there is an associated high grade fever, sore throat, and erythematous skin rash.1,2 The course is usually benign with resolution in a few months with the use of non-steroidal anti-inflammatory drugs. This condition was first described independently by Kikuchi and Fujimoto in 1972.3,4 As far as I know, the association of Kikuchi-Fujimoto disease with small and large joint polyarthritis has not been described before.
A 22 year old woman from western Nepal presented with a high grade fever, severe generalised joint pains, sore throat, and rashes for five weeks. On examination she looked ill and had a temperature of 105°C. She had painless cervical and inguinal lymphadenopathy, significant synovitis of symmetrical large and small joints, including distal and proximal interphalangeal joints, and a circumscribed erythematous scaling rash on chest, back, hairline, and legs. No organomegaly was present. Respiratory and cardiovascular examination were normal.
Investigations showed a daily spiking temperature of 105°C, erythrocyte sedimentation rate 125 mm/1st h, packed cell volume 0.28, raised white cell count 13.3×109/l, aspartate aminotransferase 283 U/l (normal<34), and alanine aminotransferase 374 U/l (normal <31). Creatinine and urine analysis were normal; urine and blood culture were negative; antinuclear antibody, rheumatoid factor, and hepatitis B surface antigen were all negative. A chest x ray examination was normal. A hand x ray examination showed periarticular osteoporosis. Lymph node biopsy disclosed histiocytic necrotising lymphadenitis. A diagnosis of Kikuchi-Fujimoto disease was made. She did not respond to two weeks' treatment with high dose aspirin and two weeks' treatment with maximum dose indometacin. A reducing course of prednisolone 40 mg was given over eight weeks, which resulted in a complete recovery.
This case is important for three reasons. Firstly, young Asian women presenting with symptoms and signs in keeping with a diagnosis of acute systemic lupus erythematosus, tuberculosis, or even adult Still's disease should have a lymph node biopsy in order to rule out the more benign condition of Kikuchi-Fujimoto disease. Secondly, Kikuchi-Fujimoto disease has been described in association with encephalitis, neuritis, and arthralgia. This is the first description of an association with extensive small and large joint synovitis. Thirdly, most patients with Kikuchi-Fujimoto disease respond well to non-steroidal anti-inflammatory drugs. This patient required a reducing dose of steroids over eight weeks in order to treat the synovitis. When this condition occurs in association with synovitis, steroids may be needed.
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