CASO CLÍNICO

 

Kimura disease: Report of a case in a Caucasian male and revision of the literature

La enfermedad de Kimura: comunicación de un caso en un varón caucásico y revisión de la literatura

 

 

J.M. López-Arcas Calleja¹, T. Martínez Iturriaga², M. Patrón Romero³, G. de María Martínez⁴,
J. González Martín-Moro⁴, J.L. del Castillo⁴

1 Médico Residente. Servicio de Cirugía Oral y Maxilofacial. Hospital Universitario La Paz. Madrid, España.
2 Médico Residente. Servicio de Cirugía Oral y Maxilofacial. Hospital Universitario Ramón y Cajal. Madrid, España.
3 Jefe de Sección Anatomía Patológica. Hospital Universitario La Paz. Madrid, España.
4 Médico Residente. Servicio de Cirugía Oral y Maxilofacial. Hospital Universitario La Paz. Madrid, España.

Dirección para correspondencia

 

 

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ABSTRACT

Kimura disease is a chronic inflammatory disorder mainly involving Asians. It often presents as soft-tissue nodes or lymphadenopathy of the head and neck. Commonly associated with increased levels of eosinophils and IgE in peripheral blood. We report a case of Kimura disease in a Caucasian, diagnosed after lymph node and submaxillary gland removal.

Key words: Kimura disease; ALHE; Eosinophil.

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RESUMEN

La enfermedad de Kimura es un trastorno inflamatorio que afecta principalmente a sujetos de origen asiático. Se manifiesta por la existencia de masas de tejido subcutáneo y subplatismal generalmente en la cabeza y el cuello. Suele acompañarse de linadenopatías, y aumento de los niveles de eosinófilos e IgE en sangre periférica. Comunicamos un caso de enfermedad de Kimura, descubierto en un varón caucasiano, tras una biopsia excisional de una masa cervical.

Palabras clave: Enfermedad de Kimura; Hiperplasia angiofolicular; Eosinofili.

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Introduction

Kimura disease is an inflammatory disorder of unknown etiology that mainly affects individuals of Asian origin. Clinically it tends to manifests as subplatysmal and subcutaneous tissue masses that are located generally in the area of the head and neck. There tends to be salivary gland and regional lymph node involvement. Histopathologically it is characterized by lymphadenopathy in the form of fibroinflammatory pseudotumors, with hyperplasia of germinal follicles, interstitial fibrosis, marked eosinophilic infiltrate of the mass and of adjacent tissue and often there will be eosinophilia and increased levels of serum IgE in peripheral blood.

We report a case of Kimura disease, discovered in a Caucasian male after an excisional biopsy of a neck mass.

 

Case report

A 35 year old Spanish Caucasian male presented with a neck mass that had been growing steadily for three weeks. On examination and palpation the existence of a large mass with a hard consistency was noted in the left submandibular region. It measured approximately 3.3 cm and there was in addition lymphadenopathy that was apparently related to the mass. FNA was carried out and the result was inconclusive, but a tumor of salivary origin was excluded. As a tumor with lymphoid lineage was suspected due to the rapid growth of the tumor, which had doubled in volume in weeks, an excisional biopsy of the mass was carried out.

The surgery was carried out with general anesthesia. After a conventional submandibular approach the existence of a large conglomeration with imprecise edges was confirmed. It contained the mass together with five to six lymph nodes measuring 1.1 cm that were adhered to it, and a large submandibular gland. The mass and the lymph nodes were excised en bloc together with the gland as it was suspected that it was also affected.

The histopathologic diagnosis was of Kimura disease. The study was later completed by means of the determination of an eosinophilic count and IgE in peripheral blood, as well as a urine test with total protein in urine, in order to evaluate the renal function of the patient.

The analysis showed considerable elevation of serum levels, of IgE and eosinophils but there was no renal function disturbance.

 

Anatomopathologic study

Macroscopic description

The lymph node measured 3.5 by 2 cm and the submaxillary gland 4 by 3 cm. The lymph gland section had a nodular aspect and marked capsular fibrosis could be observed. The submaxillary gland did not display any macroscopic disturbance (Fig. 1).

Microscopic description

The lymph gland had conservative architecture with intense reactive follicular hyperplasia and some follicles indicative of folliculosis could be observed. The capsule showed marked fibrosis that extended towards the adjacent lymph node parenchyma (Fig. 2). The lymph tissue as well as the capsule showed marked eosinophilic infiltration. These in turn formed microabscesses in the interior of the lymphoid tissue (Fig. 3), while in the capsule they had a mainly perivascular distribution. Vasculitis was not observed (Fig. 4). Nor was tumor infiltration observed at any point. IgE deposits were shown in the follicle centers through immunohistochemical techniques. Specific techniques were not able to reveal the presence of parasites (Fig.5). The salivary gland sections sent away showed only small lymphocytic infiltrates with some periductal infiltration of eosinophils. These ducts sometimes appeared slightly dilated.

 

Discussion

Kimura disease was first described in the Chinese literature in 1937,¹ but its definitive histopathologic description was not until 1948 (Kimura el al.).² It is endemic in Asia (especially in China and Japan), but it is extremely uncommon with 200 cases identified in Asia since its histological description, and it has been identified sporadically in the rest of the world. There is a male predominance and the peak incidence is in the third decade. With regard to location, various series³ have confirmed that the most common location is the head and the neck (76%), also the orbital region, eyelids, palate and pharynx, and peripherally the arms and armpits.^3,6 Clinically Kimura disease tends to be a localized process, simulating on occasions a neoplasm, as occurred in our case. Cases with systemic clinical signs have been described^6,7 in the form of pruritus, eczema, rashes etc., but this is less common and its association with renal pathology is more common (approximately 50% of cases), generally in the form of extramembranous glomerulonephritis that can develop before the growth phase of the neck mass, but this did not occur in our patient.

One of the principal problems with Kimura disease is the difficulty diagnosing it. Not only is it an extremely rare entity in the general population, but it requires a precise clinico-pathologic diagnosis in order to distinguish it from other entities with similar characteristics such and Hodgkin’s lymphoma, Castleman’s disease, dermatopathic lymphadenopathy, Churg- Strauss allergic granulomatosis, parasitic lymphadenitis and ALHE. In our case, only the anatomopathologic study of the sample permitted the diagnosis as the cytology was totally unspecific.

In fact there has been much confusion in the literature with the latter. Initially it was considered that ALHE as well as Kimura disease were clinically similar, and that they represented two varieties of the same clinicopathologic entity. It was not until 1979 that both entities were clearly differentiated, with ALHE being encompassed in the histiocytoid hemangioma group. The differences between both entities are superficially exposed in the following table.

The etiology behind Kimura is still unknown. It has been postulated that it could be an autoimmune disease.⁹ The evidence of peripheral eosinophilia, the increase in mastocytes and increased levels of IL-5 suggest abnormal stimulation of T lymphocytes similar to hypersensitivity reactions. The considerable follicular hyperplasia could be suggestive of a possible state of immunodeficiency and VH-8 infection, however, it has not been possible to demonstrate this.¹⁰

With regard to treatment for Kimura disease, it should be pointed out that surgery constitutes the main treatment with corticotherapy, radiotherapy and even chemotherapy being alternatives. In spite of the limited postoperative follow- up period (10 months) the patient is currently free of disease. Recurrence of the disease after any of these treatments is quite common.

 

Conclusions

Kimura disease is an inflammatory disease that is characterized by intense eosinophilic infiltration of the lymph nodes and sometimes of subcutaneous cellular tissue. Epidemiologically it mainly affects males of Asiatic origin. It is most commonly located in the head and neck and the maxillofacial surgeon should keep this in mind.

 

 

Dirección para correspondencia:
Dr. José María López-Arcas
Servicio Cirugía Maxilofacial Hospital Universitario La Paz
Paseo de la Castellana s/n, 28017 Madrid, España
Email: drlopezarcas@tiscali.es

Recibido: 07.02.05
Aceptado: 06.10.06

 

 

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