Annals of PIMS-Editorial Board

Acute Necrotizing Lymphadenitis

INTRODUCTION

Kikuchi disease, also known as “acute necrotizing lymphadenitis” or Kikuchi-Fujimoto disease, causes lymph node inflammation. It is a rare self limiting disease effecting people of all ethnic background. Although any age group can be affected, yet it seems to affect young adults, particularly women about 3 times more often than men. . The first case reported outside Japan was in 1982.¹ Its exact etiology is not yet known, although some researchers have suggested it is an infection or autoimmune disorder It has been linked to sequelae of infection by human herpes virus 6, cytomegalovirus (CMV), and even human T-lymphotropic virus. 1.^2-5 Recent reports had suggested links between Kikuchi’s disease and HHV-8 or Epstein-Barr virus (EBV). These, however were disregarded by George et al.⁶

CASE REPORT

The patient was a 17 years old female resident of Haripur admitted through OPD with the complaint of swelling over right side of her neck for 6 days back. It was solitary, painful, mobile, and gradually increased in size, reaching a maximum of 2.5 x 2cms in 3 days. It was accompanied with fever which was initially low grade intermittent but as the swelling increased in size, the fever also became high grade and continuous. It was associated with generalized body aches, pains, prostration and occasional headache. She suffered from almost 6 to 7 similar episodes of cervical lymphadenopathy and fever in the last 5 months. For the same complaint she was once admitted in Agha Khan University Hospital and was extensively worked up including the C.T scan and FNAC, but results were inconclusive. She was discharged without a confirmed diagnosis.
On   general physical examination, this young girl was ill looking and toxic. She had a fever of 104^oF, was markedly pale, and had dry mucous membrane and dry coated tongue. There was an enlarged anterior cervical lymph node 2.5x2 cms in size. It was solitary, extremely tender, mobile, having no discharge. No other accessible lymphadenopathy was noted. Systemic examination also did not reveal any abnormality.          Based on clinical history and examination, diagnosis of acute suppurative lymphadenitis was made. A viral etiology was also considered. Her blood complete picture showed Hb of 9 g/ dl, TLC of 3600/cmm and normal differential. ESR was 115 mm at the end of one hour. Urinanalysis was normal. Immediate blood culture was sent on admission which did not grow any organism. Anti nuclear antibody and RA factor were negative. Montoux test was positive. ECG, ultrasonography of the abdomen, liver function tests, renal function tests, electrolytes, and X-ray chest all did not show any abnormality. CT Scan neck showed enlarged lymph nodes bilaterally, the largest being 18 mm on left side. Another lymph node (12mm) on left side just superior to the thyroid gland showed peripheral enhancement and central necrosis. C.T. abdomen, pelvis and bone marrow were unremarkable. Bone marrow aspiration did not show any evidence of leukemia, lymphoma or granuloma. Excisional lymph node biopsy showed marked necrosis of lymph nodes, extensive nuclear fragmentation and phagocytic debris, with no neutrophilic infiltrate, caseastion necrosis or granulomas.          Final diagnosis of   Acute Necrotizing Lymphadenitis (Kikuchi’s Disease) was made. She was started on NSAID and nutritious diet. Adequate hydration was maintained and she gradually recovered within six weeks.

Discussion

Kikuchi’s disease is an idiopathic illness characterized by generally benign and self-limited course. Lymphadenopathy most often resolves over several weeks to 6 months, although the disease occasionally persists longer. The disease recurs in about 3% of cases.