| Summary: | Background: Kikuchi disease (KD) or histiocytic necrotizing lymphadenitis is a benign self-limited extremely-rare disorder of unkown etiology. It is characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. The recognition of KD is crucial as it can be mistaken for or be associated with systemic lupus erythematosus (SLE).
Case report: We present a 29-year-old Croatian female admitted to the Hematology and Oncology Department, General Hospital Dr. Josip Benčević, Croatia in a life threatening condition. She was feverish (40 °C) with chills and weight loss. On clinical examination the patient had bilaterally enlarged cervical, axillary and inguinal lymph nodes with sizes up to 3 cm. Our differential diagnosis was SLE, lymphoma, sarcoidosis, Still’s disease, hemophagocytic syndrome and KD. An extensive workup was done to confirm one of these diagnoses. Methylprednisolone 100 mg iv (1.5 mg/kg) was initiated for 5 days and as the patient’s condition was severe, steroids were maintained. Lymph node biopsy histopathology was compatible with KD. Antinuclear antibody and anti-double-stranded-DNA were positive. The patient fulfilled the classification criteria for SLE. A diagnosis of SLE associated with KD was held. On CT scan there was bilateral pleural effusion and ascites. Brain MRI was compatable with lupus cerebritis. On steroids plus hydroxychloroquine the patient remarkably improved and remained in remission after 3 months.
Conclusion: Prompt diagnosis and treatment with steroids may save the life of SLE patients with KD and leads to a favorable outcome. Raising the awareness towards this possibly serious association is important.
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