Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disorder, characterized by regional lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. The cause of Kikuchi disease is unknown, although infectious and autoimmune etiologies have been proposed. The most favored theory proposes that Kikuchi disease results when one or more unidentified agents trigger a self-limited autoimmune process.

A 43-year-old female presented with acute-onset high-grade fever for 30 days associated with cervical painful swallowing, malar rash, pruritic maculopapular rash on bilateral hands and hair loss. She denies to suffers from any medical illness or history of connective tissue diseases in her family. On clinical examination, she had bilateral cervical and supraclavicular lymph node enlargement.

Laboratory finding CBC with anemia and leucopenia (RBC 3.7x10⁶, Hgb 9.8g/dl, MCV 87fl, WBC 2.7x10³), Urine examination was negative for proteinuria, biochemical test was in range, PCR 27mg/dl (≤0.5mg7dl). The tumor markers were negative and the PCR for EBV, CMV and mycobacterium tuberculosis was negative. We ruled out all infection disease. Immunological findings: ANA 1:640 positive, ds-DNA positive, anti-Sm positive, the complement titer was in range. Immunophenotype of peripheral blood and myelogram was in range.

On ultrasonography the patient show enlarged multiple right and supraclavicular lymph nodes measuring up 20–118mm. PET-CT was suggestive of multiple heterogeneously enhancing bilateral and supraclavicular lymph nodes.

Lymph node biopsy showed proliferation of lymphocytes and histiocytes inter follicular with imagine of karyorrhexis, immunohistochemical staining showed the expression of CD60 and CD163 at the population of histiocytes and CD3, CD4, CD8 at the population of lymphocytes T. germinative center CD 20+, bcl-. All the finding was characteristic of KFD.

The patient was diagnosed with SLE and KFD and we started the treatment with glucocorticoid 0.5mg/kg and hydroxychloroquine 400mg daily and she responded well to treatment with improvement of clinical sing, reduce of lymph node and inflammatory biomarkers.

The real cause of KFD is still unknown but this disease is thought to be a hyperimmune response to infections and autoimmune disease. There are a rare association of KFD that precedes SLE. The confirmation of diagnosis is done by lymph node biopsy immunohistochemical examination and immunological test for SLE.