Case Report: An unusual clinical presentation of Kikuchi’s disease: a case report

Introduction

Kikuchi’s disease is a rare disease that was originally reported in patients of Asian heritage. It usually occurs in 8–16 year old aged children and presents with fever accompanied with lymphadenopathy especially in posterior cervical region. The diagnosis is made through lymph node biopsy¹. Here we report a case of superior vena cava obstruction as an unusual presentation of Kikuchi’s disease in a 9 year old Indian girl.

Case history

A 9 year old girl presented with fever of 8 days duration with poor appetite, nausea and occasional headache. She was a previously healthy child with no significant past medical or family history. She had facial puffiness and swellings in the neck noted two days previously. There was no history of decreased urine output, vomiting, rash, arthralgia, breathlessness, skin or mucosal bleeds, significant weight loss or contact with a case of tuberculosis. On physical examination, she was febrile (102°F) and had marked periorbital edema, along with multiple, enlarged and tender lymph nodes in the posterior cervical region with the largest node measuring 3 cm. There was no rash, bleeding manifestations or enlargement of other groups of lymph nodes. Examination of the gastrointestinal, respiratory, cardiovascular and nervous systems was normal. Investigations revealed the following: hemoglobin 10.1 g/dL, white blood cell (WBC) count 2760 cells/mm (neutrophils-55%, lymphocytes-41%) with platelet count of 82,000 cells/cu.mm, erythrocyte sedimentation rate (ESR) – 50 mm/hr and C-reactive protein (CRP) – 44 mg/dL. Renal and liver function tests were normal. Blood Widal and Dengue NS1 antigen tests were negative. She was started on cefotaxim (1 g intravenously q8 hourly). The patient’s condition worsened on the second day of admission with progressive dyspnea and increasing facial puffiness and she also complained of a throbbing headache. She had venous congestion of the head and neck region with conjunctival congestion. Since chest X-ray showed mediastinal widening, the possibility of enlarged mediastinal lymph nodes causing SVC obstruction was considered and she was scheduled for a CT chest examination but she developed multiple seizures at that time. Repeat investigations on the same day (day 2 of admission) showed a fall in hemoglobin (9.6 g/dL) and platelets (67,000 cells/mm³) from the baseline. Lumbar puncture findings were normal. Two hours later, she also developed pericarditis with pericardial effusion, went into cardiogenic shock and required adrenaline support (0.5 mcg/Kg/min continuous intravenous infusion). A cervical lymph node biopsy done showed necrosis with karyorrhexis, histiocytic infiltrates, crescentic plasmacytoid monocytes, and absence of neutrophils – a picture consistent with Kikuchi-Fujimoto disease. Her blood, CSF, urine cultures were sterile. Bone marrow aspiration studies were normal. Investigation also showed ANA-IF – 3+ positivity and anti-dsDNA was strongly positive. Epstein-Barr virus (EBV) DNA PCR was negative. In view of the child fulfilling four of the ARA criteria for systemic lupus erythematosus (SLE) (viz., serositis, seizures, hematological abnormalities and ANA positivity)⁸, a diagnosis of SLE with Kikuchi disease presenting as superior vena cava syndrome was made and was treated with intravenous methylprednisolone (600 mg over 30 minutes) for 3 days. The patient showed a dramatic improvement with treatment, both clinically as well as with laboratory parameters. Her general condition improved, congestion of the face and neck decreased, the lymph nodes decreased in size and she became afebrile and remained so thereafter. After 3 days of intravenous methylprednisolone, she was changed over to oral prednisolone (1 mg/Kg/day) and oral hydroxychloroquine (5 mg/Kg/day). She was discharged after 1 week with advice to continue prednisolone and hydroxychloroquine (at the same dose). At the time of follow up (after 2 weeks) she remained asymptomatic, blood count and disease activity were normal and she did not develop any features of steroid toxicity and had no evidence of adrenal insufficiency.

Discussion

To the author’s knowledge, this is the first report of SVC obstruction as a clinical presentation of Kikuchi’s disease (due to enlarged mediastinal lymph nodes).

The etiology of Kikuchi’s disease is not entirely known. It has been linked to sequelae of infection by human herpes virus 6 (HHV-6), cytomegalovirus (CMV), EBV and even human T lymphotropic virus 1 (HTLV-1)^2–4. Serological tests conducted with our patient ruled out the possibility of an associated infection. The most common clinical presentation of Kikuchi's disease is fever and cervical lymphadenopathy in a previously healthy young woman⁵. In a literature review describing 244 patients affected by Kikuchi’s disease, the common presentations were fever, fatigue, and joint pain with cervical lymphadenopathy, leucopenia, elevated ESR, and anemia⁶. Our patient also had prolonged fever with constitutional symptoms and cervical lymphadenopathy with raised ESR.

Our patient also presented with features of SLE (she fulfilled four of the ARA criteria for SLE viz., serositis, seizures, hematological abnormalities and ANA positivity). According to Kucukardali et al.⁶, SLE-associated Kikuchi’s disease is more common in cases from Asian countries than from Europe. Among the 28 cases studied by Kucukardali et al., 18 cases presented with both SLE and Kikuchi’s disease diagnosed simultaneously, 6 cases were diagnosed with SLE after being diagnosed with Kikuchi’s disease and 4 were previously diagnosed with SLE⁶. The clinical features of SLE and Kikuchi’s disease are very similar and definite discrimination between them is based on histopathological findings⁷. The absence of hematoxylin bodies and paucity of neutrophils indicate Kikuchi’s disease rather than SLE and our patient showed definite features of Kikuchi’s disease in the lymph node biopsy.

Conclusion

Kikuchi’s disease should be considered in any child, especially from Asian heritage, who presents with the typical clinical features, as prompt diagnosis of this condition will avoid further unnecessary investigations. An unusually severe form of Kikuchi’s disease can have a presentation similar to SVC obstruction syndrome due to enlarged mediastinal lymph nodes. Hence timely recognition of this condition and prompt institution of steroid therapy will result in a dramatic clinical response and a life saving measure.

Consent

Written informed consent for publication of clinical details was obtained from the child’s parents.