Kikuchi Fujimoto's Disease Appearing as Systemic Lupus Erythematosus

Abstract

A 22-year-old woman with no personal history came to the hospital due to fever and cervical lateral adenopathy which started 15 days prior. Laboratory tests show leukopenia 2800 per mm3 (with no lymphopenia), normocytic, normochromic anemia (hemoglobin 11 g/dl, hematocrit 34%, mean corpuscular volume 85.9, mean corpuscular hemoglobin 28.6 pg) and ESR 85 mm/h. The blood chemistry and urine sediment shows no abnormalities. HIV, B and C hepatitis, toxoplasma, rubeola, Epstein–Barr virus and cytomegalovirus were negative. Blood cultures and Mantoux tests were negative. Autoimmunity tests showed positive ANA with a 1/320 titer (homogeneous pattern), negative antiDNA, negative rheumatoid factor and normal complement levels. Thorax and abdominal CT showed multiple cervical and inguinal adenopathies. A bone marrow aspirate-biopsy was performed, as well as cultures, which were all negative. A cervical lymph node biopsy was performed, showing paracortical necrosis with cell detritus and numerous hystiocytes, compatible with necrotizing lymphadenitis or KFD (Fig. 1). In the following years the patient remained stable, with no further episodes of fever, although she did present self-limited cervical adenopathy. She repeatedly presented painful oral ulcers and further analysis confirmed leukopenia (without lymphopenia). Three years later she presented painful erythematous nodules on the forehead and cheeks (Fig. 2). Lab-