|Year : 2014 | Volume
| Issue : 1 | Page : 77-79
Steroid therapy in Kimura's disease
MV Archana1, K Varadaraj Shenoy1, Elizabeth Varkey Cherian1, Kishan Shetty2
1 Department of Paediatrics, Father Muller Medical College and Hospital, Kankanady, Mangalore, Karnataka, India
2 Department of Pediatric Surgery, Father Muller Medical College and Hospital, Kankanady, Mangalore, Karnataka, India
|Date of Web Publication||15-Mar-2014|
K Varadaraj Shenoy
Department of Paediatrics, Father Muller Medical College and Hospital, Kankanady, Mangalore - 575 002, Karnataka
Source of Support: None, Conflict of Interest: None
Kimura's disease is a rare condition and generally presents as non-tender subcutaneous swellings in the head and neck region, primarily seen in young Asian males. We report a case of Kimura's disease, who presented to us with swelling in the head and neck region, histopathological examination confirmed the diagnosis. Investigations showed eosinophilia and raised immunoglobulin E levels. The swelling which recurred after excision subsided following short course of steroid therapy.
Keywords: Eosinophilia, immunoglobulin E levels, Kimura′s disease
|How to cite this article:|
Archana M V, Shenoy K V, Cherian EV, Shetty K. Steroid therapy in Kimura's disease. Muller J Med Sci Res 2014;5:77-9
| Introduction|| |
Kimura's disease is a rare benign chronic inflammatory disorder of unknown etiology, primarily seen in young Asian males.  It is characterized by the triad of painless unilateral cervical adenopathy or subcutaneous masses predominantly in the head or neck region, with blood and tissue eosinophilia and markedly elevated serum immunoglobulin E (IgE) levels.  Classic histopathologic features include numerous lymphoid follicles, mixed inflammatory infiltrate composed mainly of eosinophils and increased amount of post-capillary venules. 
| Case Report|| |
This was a case report of a 15-year-old boy presented to our hospital with h/o swelling in right side of neck and right preauricular region since last 4 years. The swelling was gradually progressive and painless. There was no h/o fever, cough, weight loss, cough, night sweats or exposure to tuberculosis. He did not have any rash, joint swelling or bone pain. Fine-needle aspiration cytology of the swelling done 2 years back in another hospital was reported as non-specific. The child was started empirically on anti-tubercular treatment (ATT), consisting of isoniazid, rifampicin and pyrazinamide which was continued for 6 months. However, the size of the swelling never regressed during ATT. 1 year later as there was no improvement ATT was repeated in another hospital, but again with no response.
On examination, child appeared healthy on general examination. A non-tender, non-fluctuant firm mass measuring 8 cm × 6 cm × 3 cm was palpable in the right prearicular area extending on to the submandibular area and one more mass measuring 3 cm × 3 cm × 2 cm was palpable in the sub mental region. No warmth or redness was noted on the overlying skin. 4-5 insignificant lymph nodes were palpable in the right side of the neck. Neither axillary or inguinal lymphadenopathy nor hepatosplenomegaly was noted. The rest of the physical examination was normal.
Laboratory data included a hemoglobin of 14 g/dL, Platelet count of 234,000/cumm and White cell count of 6900: Differential showed 33% eosinophils, 37% neutrophils, 26% lymphocytes and 4% monocytes respectively. The erythrocyte sedimentation rate and peripheral smear were normal. Ultrasonogram of the swelling done showed heterogeneous mass arising separately and superficial to parotid gland. Histopathology examination of biopsy specimen showed fibrocollagenous and fibrofatty tissue with proliferating lymphoid tissue in the form of follicles with prominent germinal centers. These follicles and surrounding stroma were infiltrated by numerous eosinophils. Some of the follicles contained thin walled blood vessels and proteinaceous deposits [Figure 1] and [Figure 2].
A diagnosis of Kimura's disease was made. Serum IgE done at this time showed elevated levels (6607 IU/ml). The clinical presentation, peripheral eosinophilia, raised IgE levels and histopathologic features confirmed the diagnosis. His renal function test was normal; there was no proteinuria. The residual swelling after excision biopsy started slowly increasing. A course of cetirizine was given which did not have any effect on the size of the swelling. As the parents wanted cosmetic improvement and as the child was reluctant to attend school, after a detailed discussion with the parents, the child was treated with prednisolone 0.5 mg/kg/day for 2 weeks, following which swelling regressed almost completely. Child has been followed-up for 3 months after discontinuation of steroids and there has been recurrence [Figure 3] and [Figure 4].
| Discussion|| |
Kimura's disease was first described in China as eosinophilic hyperplastic granuloma in 1937 by Kim and Szeto.  However, the entity became more widely known as Kimura's disease after a systematic description in 1948 by Kimura in Japan. 
Kimura disease has been mostly described in adolescent and adult males and there are very few reports of the disease in children. ,
Kimura's disease is characterized by gradually progressive, painless subcutaneous masses of head and neck region with lymphadenopathy. It most commonly involves soft-tissue of the neck, cervical lymph nodes; parotid and submandibular salivary gland and may rarely involve orbit, scalp and auricle. The causes and pathogenesis of Kimura's disease remain largely unknown. It is proposed that allergic or autoimmune response to unknown stimuli is responsible for the disease. Immunohistochemical studies in Kimura disease have shown marked proliferation of CD4 cells of Th2 phenotype which increase the levels of cytokines such as interleukin 4 (IL4) and IL5 which in turn can cause elevated levels of IgE and eosinophilia. ,,
The histopathology of Kimura's disease is characterized by preserved nodal architecture, prominent germinal centers in involved lymph nodes containing cellular, vascular and fibrous components. The cellular component consists of dense eosinophilic infiltrates in a background of abundant lymphocytes and plasma cells, eosinophilic microabscesses with central necrosis, Warthin-Finkeldey-type polykaryocytes, some degree of vascular proliferation of germinal centers, increased post-capillary venules in the paracortex and sclerosis. Immunoperoxidase studies show IgE reticular network in germinal centers. ,,,
Though Kimura's disease is a benign self-limiting disease, occasionally children might develop renal complications. Proteinuria occurs in 12-16% cases. , Nephrotic syndrome is the most common presentation. Renal biopsy has showed mesangial proliferative glomerulonephritis, minimal change disease and membranous glomerulonephritis. The nephrotic syndrome of Kimura disease has shown a relapsing trend, but has responded to steroid treatment. 
Differential diagnosis of Kimura's disease include angiolymphoid hyperplasia with eosinophilia, Hodgkin lymphoma, Langerhans cell histiocytosis, Castleman disease, allergic granulomatosis of Churg and Strauss, drug reaction and parasitic lymphadenitis. ,
There is no consensus on management of Kimura's disease and various treatment modalities have been tried with variable success. The primary treatment for Kimura's disease is surgical resection. Additional medical therapy including regional or systemic steroid therapy, cytotoxic therapy, antihistamines and local radiation therapy have been tried. ,, Recurrence following therapy is common and can occur in 60% in children. 
In the child reported here there was a recurrence following surgery which is common. Children treated with steroids also have recurrences. , The period of observation is too short in our report to come to any definite conclusion regarding recurrence. Cetirizine has been used in Kimura disease as it has properties of inhibition of eosinophil chemotaxis and allergen induced eosinophil influx in human tissues. We used it in our patient but without much benefit. Cetirizine was used after steroid therapy with good effect in the case report by Ben-Chetrit et al. 
| Conclusion|| |
Pediatricians have to consider this entity while dealing with painless neck masses without overt signs of other diseases. Biopsy finding with eosinophilia and elevated IgE levels will confirm the diagnosis. Excision of the mass alone or a combination of excision followed by steroid therapy in case of recurrence may be the best modality of treatment of this disease. The prognosis is good with minimal complications.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]