|Year : 2015 | Volume
| Issue : 4 | Page : 1031
Kimura's disease embedding radial artery: A very rare presentation
Komal Sawaimul, Banyameen Iqbal, Tushar Kambale
Department of Pathology, Dr. Dnyandeo Yashwantrao Patil Medical College and Research Center, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||15-Feb-2016|
Department of Pathology, Dr. Dnyandeo Yashwantrao Patil Medical College and Hospital, Pimpri, Pune - 411 026, Maharashtra
Source of Support: None, Conflict of Interest: None
Kimura's disease (KD) was first described in 1937 by Kimm and Szetoas a reactive, self-limiting, painless, persistent lesion mimicking neoplasm and described it as eosinophilic hyperplastic lymphogranuloma. The present nomenclature was given by Kimura et al., in 1948. It occurs most often in young and middle-aged Asian males. It is most common in head and neck region, involvement of peripheral vasculature although very rare has been documented.
Keywords: Benign neoplasm, eosinophilia, kimura disease
|How to cite this article:|
Sawaimul K, Iqbal B, Kambale T. Kimura's disease embedding radial artery: A very rare presentation. J Can Res Ther 2015;11:1031
| > Introduction|| |
Kimura's disease (KD) is an uncommon finding, which was first described in 1937 by Kimm and Szetol as a reactive, self-limiting, painless, persistent, indolent lesion mimicking neoplasm, and being of unknown etiology, when they reported seven cases with the term "eosinophilic hyperplastic lymphogranuloma".  The present nomenclature was given by Kimura et al., in 1948, who noted the vascular component and described it as an "unusual granulation combined with hyperplastic changes in lymphoid tissue". 
| > Case report|| |
We are presenting a case of a 22-year-old Asian male patient who presented in our hospital with a painless, soft tissue swelling in his right wrist for the last 2 months. On physical examination, he had a mobile, pulsatile, mass of approximately 3 × 2 cm diameter in the volar aspect of his right distal forearm near the radial artery. Hand was warm, other peripheral pulsations were normal. There were no associated dermal or intradermal papules, plaques, or nodules. Past medical history was noncontributory. There was no history of trauma or previous surgery of the hand. His blood counts showed eosinophilia (33%); chest X-ray and skeletal surveys were normal. Skin tuberculin test was negative. Urine and stool analyses did not show any abnormality. Serum IgE was 1,017 IU/ml (<180 IU/ml). Subsequently, an excisional biopsy was performed and sent for histopathological examination. Gross examination of the specimen showed a soft tissue/vascular piece of tissue measuring 3 × 2 cm, grey white in color, and firm in consistency. Microscopic examination showed nodular proliferation of inflammatory cells separated by fibrous tissue [Figure 1]. Lymphoid cell proliferation was predominantly seen with prominent lymphoid follicles. Sections also show predominance of eosinophilic infiltrate with a few giant cells [Figure 2]. Vascular component is sparse with arterial vessels showing thrombotic cellulitis. Diagnosis confirming KD embedding radial artery in the deep lymph glands which are chiefly grouped in the axilla, although a few may be found in the forearm, in the course of the radial, ulnar, and interosseous vessels, and in the arm along the medial side of the brachial artery.
|Figure 1: Micrograph shows nodular proliferation of inflammatory cells separated by fibrous tissue (hematoxylin and eosin (H and E), ×20)|
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|Figure 2: Micrograph showing high power view of aggregates of lymphoid cells along with dispersed eosinophils (H and E, ×40)|
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| > Discussion|| |
KD is also called as eosinophilic granuloma of soft tissue. Eosinophilic hyperplastic lymphogranuloma, eosinophilic lymphofolliculosis, eosinophilic lymphofollicular granuloma, and eosinophilic lymphoid granuloma are some of the other synonyms of this rare disease. , It occurs most often in young and middle-aged Asian males (male: female = 3.5:1).  It is most common in head and neck region, involvement of peripheral vasculature although very rare has been documented in axillary and inguinal nodes.  The etiopathogenesis of KD remains unknown and it is considered nowadays as an allergic disease and it seems to be a systemic immunological disorder. Eosinophilia and increased serum IgE levels make KD be considered a CD4 (+) T helper 2 (Th2) allergic reactions. Th2 cells would produce interleukins (IL)-4, IL-5, and IL-13; which, in turn, would act on B cells favoring the production of antigen-specific IgE. Th2 cell proliferation and the over expression of cytokines would play an essential role in the development of the disease.  In the past, there has been a quite misunderstanding that KD and angiolymphoid hyperplasia with eosinophiliaare in fact two separate disease entities. Nowadays the difference is clear; although both diseases are of unknown cause, KD is thought to be a consequence of autoimmune/allergic response to an unknown antigen, whereas, angiolymphoid hyperplasia with eosinophilia is abenign vascular neoplasm.  Malignancy should be ruled out first. The absence of Reed-Sternberg cells helps to exclude Hodgkin's disease. Unfortunately, T cell lymphomas can present with polymorphonuclear lymphocytes and eosinophilia, making the distinction difficult.  Although atypical, histiocytosis-X can present with subcutaneous masses, the diagnosis is made by finding the characteristic abnormal histiocytes  and detecting CD1A marker. Common sites of involvement are the parotid glands and the epitrochlear, axillary, and inguinal nodes. Although the masses enlarge slowly, patients remain asymptomatic otherwise. Pruritus and dermatitis may occur, and rare sites of involvement include the kidneys, orbits, ears, spermatic cord, and nerves and vessels. Common type of renal involvement is nephrotic syndrome. , Although spontaneous resolution has been reported, most patients have a prolonged course with slow enlargement of the masses.  Surgical excision of lesion(s) is the first-line therapy, even though relapses are frequent.  Systemic corticotherapy with prednisone is indicated for relapses, but with a risk of relapse when the treatment is withdrawn.  Radiation treatment is usually used for the local control of lesions not responsive to steroids. Other treatment modalities are intralesional administration of steroids, cytotoxic agents, and electrodessication. Pranlukast, a leukotriene receptor antagonist, and cetirizine, an H1 receptor blocker, were also effective in inducing clinical remission in a few cases. 
| > Conclusion|| |
KD is a rare chronic inflammatory disorder of unknown etiology that is most common in middle-aged Asian men. It involves the deep subcutaneous tissues and lymph nodes of head and neck region and rarely the extremities as in our case, with associated regional lymphadenopathy. Histopathological examination is diagnostic. Laboratory findings reveal elevated serum IgE levels and peripheral blood eosinophilia. KD has a benign indolent course which resolves over time.
| > References|| |
Kimm HT, Szeto C. Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz′s disease. Proc Chin Med Soc1937;329.
Kimura T, Yoshimura S, Ishikawa E. On the unusual granulation combined with hyperplastic changes of lymphatic tissues. Trans Soc Pathol Jpn 1948;37:179-80.
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[Figure 1], [Figure 2]