British Journal of Radiology (2003) 76, 656-658
© 2003 British Institute of Radiology
doi: 10.1259/bjr/67899714
Necrotizing cervical lymphadenopathy caused by Kikuchi–Fujimoto disease
M J Bennie, MRCP, FRCR
1
K M Bowles, MRCP
2 and
S C Rankin, MRCP, FRCR
1
1 Department of Radiology, Guy's and St Thomas NHS Trust, Guy's Hospital, London SE1 9RT and 2 Department of Haematology, Addenbrookes Hospital, Cambridge, UK
Correspondence: Dr M J Bennie, Department of Radiology, Norfolk and Norwich University Hospital, Colney Lane, Norwich NR4 7UZ
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Introduction
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A 22-year-old female presented with fever and tender right sided neck lumps. She reported generalized neck pain and was unable to fully flex, extend or rotate her neck. Initially only one lump was palpable which increased in size over a 2 week period before other lymph nodes lower down in the cervical chain became enlarged. Her General Practitioner had initially treated her with antibiotics for a presumed upper respiratory tract infection, with some improvement in her symptoms, but the nodes persisted. On direct questioning she denied weight loss and night sweats. There was no history of tuberculosis (TB) or TB contacts. She was born in the Indian subcontinent and had had a BCG as a child. She had no other medical problems.
On examination a small BCG scar was present over the left shoulder. There was an enlarged node in the upper cervical region on the right measuring 2 cm in diameter with smaller glands approximately 1–2 cm in diameter in the posterior triangle of the neck. No other nodes were palpable. The right tonsil was enlarged. The cardiovascular, respiratory and abdominal examination was normal. On investigation she was found to be leukopenic with a white blood count of 3.3 x 109/l and lymphopenic with lymphocytes of 1.09 x 109/l. Her haemoglobin was 11.2 g dl-1, erythrocyte sedimentation rate (ESR) 57, Paul Bunnel test negative, blood cultures negative, throat cultures negative and Heaf test grade 2.
Chest radiograph was normal. A CT scan of the neck during the infusion of intravenous contrast showed extensive right cervical adenopathy extending from the angle of the jaw downwards displacing the right submandibular salivary gland anteriorly. The nodes showed non-uniform enhancement with central low attenuation necrotic areas (Figure 1
). Some nodes enhanced more uniformly (Figure 2). There was no evidence of inflammatory changes within the subcutaneous tissues or skin. In view of the CT appearances, the patient's age and origin, the initial radiological diagnosis suggested was TB (Figure 3).
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Figure 1. Contrast enhanced CT scan of the neck showing enlarged right cervical lymph nodes with central areas of low attenuation necrosis.
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Figure 3. Contrast enhanced CT scan of the neck in another young patient with enlarged necrotic cervical nodes, which was histologically confirmed to be tuberculosis. The appearances are indistinguishable from those of Kikuchi–Fujimoto disease in Figure 1 .
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A fine needle aspiration of the largest of the right cervical lymph nodes showed a cellular aspirate with a mixed population of mature and immature lymphoid cells suggestive of reactive lymphadenopathy. There was no evidence of granulomata. The acid–alkali fast bacilli (AAFB) and bacterial culture was negative. An excision biopsy of the largest lymph node was performed 6 weeks later and the histological features made the diagnosis of Kikuchi disease. The patient went on to make a spontaneous and complete recovery and remained well on review in clinic 4 months later.
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Discussion
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Kikuchi disease is a form of necrotizing lymphadenitis with characteristic histological appearances, which was first described in 1972 independently by Kikuchi and Fujimoto [1, 2]. Although first descriptions were in people of Asian origin the disease has now been reported in individuals of all races. Its true incidence is unknown. An increased incidence in females is reported with a female to male ratio between 1.1:1 and 2.75:1 [3]. The age range is wide (11–80 years of age) but the majority of patients are under 30 years of age. Clinically cervical lymph nodes are the principal site of disease and are reported in 70–98% of patients. Involvement of most other nodal areas has been described including axillary, thoracic, abdominal and pelvic. Splenomegaly has also been described. Fever is the primary symptom in 30–50% of cases. Less common manifestations include weight loss, chills, skin rash, gastrointestinal symptoms and night sweats. The results of laboratory investigations are often normal. The pathogenesis is unclear but a viral or post viral hyperimmune reaction has been suggested as a possible mechanism. Autoimmune studies are usually negative, however cases associated with systemic lupus erythematosus (SLE) and Hashimoto disease have been reported. Complete spontaneous recovery is usual.
Kikuchi disease is most commonly mistaken for malignant lymphoma. In a study by Dorfman and Berry 30% of 108 lymph node biopsies reviewed were initially misdiagnosed as lymphoma [4]. The pathological diagnosis of Kikuchi disease is based on the presence of variable degrees of necrosis in the cortical or paracortical areas of a moderately enlarged lymph node with prominent cellular debris, proliferation of histiocytes and immunoblasts surrounding the area of necrosis and absence of granulocytes and plasma cells [4]. The pathological features may mimic lymphoma in some cases owing to the variable degree of microscopic necrosis found within the lymph nodes as well as the atypical appearance of the histiocytes, which may be confused with the small and large cleaved follicular cells seen in some cases of lymphoma. However, the absence of granulocytes and plasma cells usually allow the differentiation of Kikuchi disease from lymphoma or lymphadenitis caused by bacteria or viruses. The morphological distinction from SLE may be problematic in some cases [4]. Spontaneous complete resolution of symptoms usually occurs within 4 months.
Kim [5] and Fulcher [6] each described a case of Kikuchi disease with diffuse homogeneously enhancing cervical lymphadenopathy. Intraparotid, supraclavicular and mediastinal adenopathy were also present in one case [5]. The CT features of homogeneously enhancing nodes without significant nodal necrosis make radiological differentiation from lymphoma impossible. Na et al investigated two cases with MRI and contrast enhanced CT [7]. One case had similar CT findings to the other reported cases whilst the other showed inhomogeneous enhancement with extensive nodal necrosis.
Our case showed unilateral enhancing lymphadenopathy with central necrosis. As with all the previous reports there was accentuation of the fascial planes and obliteration of the perinodal fat. This case highlights the variability of the radiological appearances of cervical lymphadenopathy with Kikuchi disease and although the most reported CT findings are of uniform nodal enhancement, central necrosis can also occur and mimic TB or metastases in the appropriate age group.
There are no specific clinical or radiological features of Kikuchi disease. Diagnosis is made on a pathological examination of an excised lymph node biopsy. However, awareness of the condition and the spectrum of CT appearances will help the radiologist suggest the disease as a possible differential diagnosis, particularly in young patients with isolated cervical lymphadenopathy.
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References
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- Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytosis. Nippon Ketsueki Gakkai Zasshi 1972;35:379–80.
- Fujimoto Y, Kozima Y, Yamaguchi K. Cervical subacute necrotising lymphadenitis: a new clinicopathologic entity. Naika 1972;20:920–7.
- Kuo T. Kikuchi's disease (histiocytic necrotising lymphadenitis): a clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology and DNA ploidy. Am J Surg Pathol 1995;19:798–809.[Medline]
- Dorfman RF, Berry GJ. Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 1988;5:329–45.[Medline]
- Kim TA, Lupetin AR, Graham C. CT appearances of Kikuchi-Fujimoto Disease. Clin Imaging 1995;19:1–3.[Medline]
- Fulcher AS. Cervical lymphadenopathy due to Kikuchi Disease: US and CT appearances. J Comput Assist Tomogr 1993;17:131–3.[Medline]
- Na DG, Chung TS, Byun HS, Kim HD, Ko YH, Yoon JH. Kikuchi disease: CT and MR findings. AJNR Am J Neuroradiol 1997;18:1729–32.[Abstract]
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J. H. Youk, E.-K. Kim, K. H. Ko, and M. J. Kim
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J. Ultrasound Med.,
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27(6):
847 - 853.
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Introduction
Discussion
