Kimura's Disease: A Clinical Case

Kimura’s disease is a benign, but locally injurious disease with a marked predilection for the head and neck. Of uncertain aetiology, its tendency to present as a discrete, enlarging mass with associated lymphadenopathy makes it a condition of interest to clinicians who see head and neck pathology. Although rare, there are increasing numbers of reports of the condition and it should become part of the standard differential diagnosis. A typical case of this rare disease is presented.

INTRODUCTION

Kimura’s disease is an unusual condition in several respects. It was first reported by Chinese authors Kimm and Szeto in 1937[1] and, besides its eponym, has been variously known as epithelioid haemangioma, atypical pyogenic granuloma and cutaneous eosinophilic lymphofolliculosis. The definitive description was published by Kimura et al in Japan in 1948[2] and since that time, there has been a slowly increasing number of reports. While most cases have originated in China, Japan or Southeast Asia, there have been sporadic case reports from Europe and America and by late 1994 there had been some 120 cases described in the world literature. The majority of patients have originated in the Eastern hemisphere, but ongoing immigration and population mixing have resulted in increased incidence in Western countries. The disease is most prevalent in Asians, uncommon in Caucasians and rare in Blacks. It has been suggested that the common factor is a degree of Asian ancestry.[1]

Kimura’s disease is a chronic inflammatory condition which presents with a characteristic triad of signs and symptoms, namely a painless, slowly enlarging soft tissue mass (or masses), associated lymphadenopathy and peripheral eosinophilia. Eighty-five per cent of cases occur in men.

Clinically, the subcutaneous soft tissue masses occur predominantly in the head and neck, and often involve the parotid glands. Approximately 67 to 100% of patients develop regional lymphadenopathy and, in longstanding disease, this may become generalized.[3] Patients may complain of local or generalized pruritus and subacute or chronic dermatitis. There may be proteinuria and laboratory investigations will invariably reveal peripheral eosinophilia and increased serum immunoglobulin (Ig)E.

While there has been considerable discussion in the pathology literature concerning this disease, it is still unknown by most surgeons. This report seeks to increase awareness of an interesting condition.

CASE REPORT

An 18-year-old male presented with a complaint of a slowly enlarging mass in the right submandibular region. History revealed that he was having this swelling since 4 months and was treated with antibiotics following which it used to subside but again appeared within 10 days of stopping antibiotic. Fine needle aspiration cytology revealed inflammatory cells and differential count revealed marked eosinophilia.As patient was not having any local infection clinically diagnosis was not reached.Hence excision biopsy was performed which revealed a dense inflammatory infiltrate and fibrosis. The infiltrate was characterized by lymphoid tissues with germinal centres and numerous eosinophils with eosinophilic microabscess forma tion. A prominent proliferation of small venule-sized vessels was noted. There was no evidence of malignancy and no organisms were noted (Fig. 1).

Fig 1 : Histology of the lesion. Note the dense inflammatory infiltrate with eosinophilia and germinal centre arrangement of the lymphoid tissue.

DISCUSSION

The differential diagnosis, while including obvious lesions such as dermatofibrosarcoma protruberans and cylindroma (turban tumours), will ultimately be determined by both the clinical picture and the histopathology. Clinically, malignant lymphoma, parotid tumours, haemangioma, pyogenic granuloma, Mikulicz’s disease and Kikuchi’s disease are all conditions for which Kimura’s disease has been mistaken in the past.[3] Other conditions to consider include Kaposi’s, sarcoma, angiosarcoma, eosinophilic lymphoma and angioimmunoblastic lymphadenopathy; parasitic diseases responsible for subcutaneous masses with an associated lymphadenopathy, such as tissue-invasive helminth infections, cysticercosis, sparganosis, toxocariasis and several forms of invasive miasis may also need to be ruled out.[1]

In Kimura’s disease there is classically a dense inflammatory infiltrate characterized by eosinophilic lymphoid tissue with germinal centres and microabscesses. There is often marked fibrosis found within the typical lesions. Pathologically, the picture is perhaps most difficult to distinguish from angiolymphoid hyperplasia with eosinophilia (ALHE), and for a long time these two conditions were thought to represent one and the same pathology, but the current consensus is that they represent two ends of a spectrum of similar diseases.[4]

In contrast to that of Kimura’s disease, the histology of ALHE is typified by an exuberant proliferation of capillary vessels with marked irregularity of luminal sizes. Lining endothelium comprises plump, vacuolated cells. While there is an inflammatory infiltrate, the associated lymphocytes are not arranged in germinal centres as they are in Kimura’s disease. Further, while ALHE is marked by the presence of histiocytoid endothelial cells, these are absent from Kimura’s lesions.

Clinically, Kimura’s disease is believed to be a disease of the Far East and Asia, and ALHE one of the western world. Jambhekar et al[4] suggest that while ALHE is known to occur in the Far East and Asia, Finally, while ALHE occurs in older, predominantly female populations, Kimura’s disease is primarily a disease of younger males as in our case where an 18 year old male is affected.

The cause of Kimura’s disease remains enigmatic and the low incidence of the condition makes clinical research in this regard difficult. There are, however, several theories of aetiology.

The eosinophilia and elevated IgE levels seem to favour a parasitic cause, but this has never been established. Somewhat more appealing is the suggestion that this entity may represent a form of unusual atopic response. Evidence for this stems form the observation that the histologic features vary with site and duration of the lesion,[5] as well as the local tissue eosinophilia, mast cell hyperplasia, polytypic lymphoplasmacytic infiltration with reactive germinal centres and a blood picture typified by eosinophilia and raised serum IgE levels. Our patient showed eosinophilia in differential leucocyte count.
The treatment of choice for localized disease is surgical excision,[1,3] although some authors make a plea for conservative management, in the form of intralesional steroids[1] or radiation therapy[6] particularly in anatomically sensitive areas such as the periorbital region.[7] There have also been reports of recurrence in 15 to 40% of cases,[7] even after apparently adequate surgical excision and excisional biopsies,[3] but this may be because of the poor delineation of the borders of Kimura’s lesions, suggesting that adjunctive frozen sections may be useful, especially in re-excision of recurrences.

In summary, Kimura’s disease is an indolent, benign, but locally disfiguring disease, whose true importance lies in its ability to mimic a number of other benign inflammatory and neoplastic conditions of the head and neck. Knowledge of the condition, its clinical appearance, course and histopathology puts the practitioner in a better position to answer questions from concerned patients and primary caregivers, and optimize management strategies.

ACKNOWLEDGEMENT

We would like to thank Dean and Dr. M.G.Rathod Head of the Department of Surgery, Grant Medical College and JJ Group of Hospitals. For granting us permission to publish this case report.

REFERENCES

1.Irish JC, Kain K, Keystone JS, Gullane PJ, Dardick I. Kimura’s disease: An unusual cause of head and neck masses. J Otolaryngol 1994;23:88-91.

2.Kimura T, Yoshimura S, Ishikawa. Unusual granulomata combined with hyperplastic change in lymphatic tissue. Trans Soc Pathol Jpn 1948;13:179-80.

3'Chih-Yau Lee, Chih-Ying Su, Shyr-Ming Sheen-Chen, Hock-Liew Eng, Wei-Jen Chen. Kimura’s disease: Report of four cases. Chang Gun Med J 1994;17:153-7.

4.Jambhekar NA, Bores AM, Saxena R, Parikh D, Soman C. Angiolymphoid hyperplasia with eosinophilia (Kimura’s disease): Report of a large-sized lesion. J Surg Oncol 1991;47:206-8.

5..Olsen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia. A clinicopathologic study of 116 patients. J Am Acad Dermatol 1985;12:781-96.

6.Itami J, Arimizu N, Miyoshi T. Radiation therapy in Kimura’s disease. Acta Oncol 1989;28:511-4.

7.Kennedy SM, Pitts JF, Lee WR, Gibbons DC. Bilateral Kimura’s disease of the eyelids. Br J Ophthalmol 1992;76:755-7.