Kimura’s Disease: A Case Report

Waqar Ahmed Ansari*, Syed Javed Arshi**, Girish Mazumdar***, Vinaya Ambore+

Abstract

An 11 year old male boy presented with painless swelling in right side of neck anterior to tragus of 4 months duration, without any history of fever, cold, cough, or any history suggestive of tuberculosis: On examination there were firm, discrete, non tender not freely mobile preauricular and upper jugular group of lymph nodes palpable, largest lymph node was approximately 2.5 cm in preauricular region, for which patient had consulted.
As tuberculosis is one of the commonest diagnosis in India for cervical lymphadenopathy, work up for the same was carried out. ESR was normal, with WBC = 8700/mm3 without any evidence of eosinophilia. Mantoux test as well as X-ray chest were negative. Ultrasound revealed multiple enlarged lymph nodes at the right angle of mandible, largest 2.5 cm, with few lymph nodes seen in the right parotid gland. A diagnostic biopsy of the preauricular lymph node performed. Histopathology was reported as Angiolymphoid Hyperplasia with Eosinophilia (Kimura’s Disease) of right pre-auricular region.

Introduction

Kimura’s disease is a rare inflammatory
disorder of unknown cause, primarily seen in young males, involving mainly subcutaneous tissue, salivary glands and lymph nodes. It was first reported in 1946.1 The disease usually presents with painless subcutaneous nodules, lymph node enlargements, blood eosinophilia, elevated levels of serum Immunoglobulin E and systemic associations such as nephritic syndrome.2 Juvenile temporal arteritis is frequently seen in Kimura’s disease.3 A rare association of Kimura’s disease, with life threatening coronary artery spasm has also been recorded in both adult as well as paediatric age groups.3 The clinical course is usually benign. Treatment options are surgical excision, follow up and corticosteroids.

Case Report

An 11-year-old boy presented with painless swelling in right side of neck, just anterior to tragus of 4-month duration. There was no history of fever, cough, cold or past history of tuberculosis. There was no h/o similar swelling elsewhere in the neck, axilla or groin. There was no history of weight loss, evening rise of temperature, anorexia or night sweat. On examination multiple preauricular and upper jugular chain of lymph nodes were enlarged. They were non tender, firm with restricted mobility, largest, among them was preauricular lymph node of 2.5 cm in size. As tuberculosis is common aetiology the boy was investigated for the same. Haemoglobin was 8.5 gm% with normal eosinophil count i.e 4%, TLC– 8700/cm, ESR– 20 mm at the end of one hour, mantoux test was negative, and X-ray chest was normal. USG of the neck showed “multiple enlarged lymph nodes at the right angle of mandible, largest 2.5 cm across. The right parotid gland was normal in size and echo texture; there were multiple enlarged lymph nodes seen within the gland of which the largest was 3 cm across. Another separate superficial preauricular lymph node 2.5 cm in size was also present. To confirm the clinico-radiologic impression, an well as to give cosmetic result (as patient has presented with cosmetic result (as patient has presented with preauricular swelling) surgical excision of preauricular lymph node was carried out.
Histopathology of the excised lymph node was reported “Angiolymphoid hyperplasia with Eosinophilia (Kimura’s disease), right neck”.
Eosinophil counts were in normal range 4%. Serum Immunoglobulin E levels could not be done. Retrospective history was taken from patient to rule out any involvement of kidney, or arteries, i.e. coronary or temporal arteritis. There was no history suggestive of any of these systemic associations.

Discussion

Kimura’s disease is a rare inflammatory disorder of unknown cause, primarily affecting young males, involving mainly subcutaneous tissue, salivary Glands and lymph nodes. It was first reported in 1946.1 It has been more commonly seen in Asian males. Classic triad of the disease is painless subcutaneous masses in head or neck region, blood and tissue Eosinophilia, and markedly elevated serum immunoglobulin level. The disease is known to be associated with systemic disease, like nephritic syndrome2 Juvenile Temporal arteritis, coronary artery spasm in adult as well as in childhood.3

Histopathology of the subcutaneous masses or lymph nodes shows Angiolymphoid hyperplasia with Eosinophilia (ALHE). Wells and Whimster first described ALHE in 1960 as a variant of Kimura’s disease.

Kimura’s disease is said to be associated with systemic disease more commonly than ALHE. It is still not clear, whether these two entities represent a spectrum of a single disease.

It is interesting to note that calcium channel antagonist or Nitroglycerine, which is commonly used for variant angina, shows little effect in coronary artery spasms associated with Kimura’s disease.4 In contrast, Glucocorticoids seem to exert strong anti-vasospastic effect on coronary artery by reducing eosinophilic infiltrations and suppressing the development of inflammatory mediators.5

It is important to diagnose the cause of lymphadenopathy. In our country, tuberculosis is common cause of lymphadenopathy. Therefore it is necessary to rule out other aetiologies.

Kimura’s disease has benign course, and does not require any long term treatment.

References

1. Pediatrics. 2002; 110 (3) : e39.
2. Altman DA, Griner JM, Lowe L, Angiolymphoid hyperplasia with Eosinophilia and nephrotic syndrome. Cutis 1995; 56 : 334–6.
3. Takahashi N, Kondo K, Aoyagi J. Acute myocardial infarction associated with hypereosinophilic syndrome in a young man. Jpn Circ J 1997; 61: 803–06
4. Hirakawa Y, Koyanagi S, Matsumoto T, et al. A case of variant angina associated with Eosinophilia. Am J Med 1989; 87 : 472–4.
5. Rothenberg Me. Eosinophilia. N Engl J Med 1998; 338 : 1592–1600.

Fig.1 : Angiolymphoid hyperplasia with Eosinophilia (Kimura’s disease) right neck (×40 power). Note endothelial lined spaces and eosinophils.	Fig.2 : Angiolymphoid hyperplasia with Eosinophilia (Kimura’s disease) of right neck (×10 power). Note lymphoid follicle surrounded by endothelial lined spaces and eosinophils.