Kimura Disease: A Rare Case Report

Microcirculatory systems, which can be divided into several segments, have crucial physiological functions. We investigated whether monoclonal antibody against alpha-smooth muscle actin (alpha SMA) was useful for the identification of microcirculatory segments, according to the structure of their periendothelial cells, in two pathological cutaneous conditions. We examined skin specimens from patients with Kimura's disease and with angiolymphoid hyperplasia with eosinophilia, since little information is available on microvascular segments in these conditions. Immunostaining for alpha SMA revealed the morphological characteristics of the periendothelial cells clearly enough to identify five segments in the proliferative microvessels of Kimura's disease and angiolymphoid hyperplasia with eosinophilia. In Kimura's disease, postcapillary venules were predominant, while each vascular segment in angiolymphoid hyperplasia with eosinophilia was uniform. Vessels without periendothelial cells were detected to a greater extent in angiolymphoid hyperplasia with eosinophilia than in Kimura's disease. The antibody against alpha SMA appeared to be useful in the observation of periendothelial cells for the identification of vascular segments in pathological cutaneous conditions.

Kimura's disease is a rare chronic inflammatory condition of uncertain etiology which has an affinity for the Asian population. It primarily involves the head and neck region, presenting as deep subcutaneous masses and is often accompanied by regional lymphadenopathy and salivary gland involvement. Peripheral blood eosinophilia and elevated serum immunoglobulin E (IgE) levels are characteristic features and the microscopic picture reveals lymphoid proliferation with eosinophilic infiltration. For years, Kimura's disease was believed to be identical to or part of the same disease spectrum as angiolymphoid hyperplasia with eosinophilia (ALHE). Recent reports, however, have confirmed that the two are, in fact, separate entities. We report a case of Kimura's disease in a 22-year-old Indian male who presented with a subcutaneous mass, parotid enlargement and lymphadenopathy. The clinical presentation was suggestive of Kimura's disease and microscopic examination following biopsy of the lesion allowed us to make a definitive diagnosis.

Rare case of Kimura disease of the upper lip: A case report

Kimura's disease (KD) is a benign chronic inflammatory disorder attributed to an immune-mediated hypersensitivity which often presents as a tumor-like swelling in the head and neck region with or without lymphadenopathy, associated with hypereosinophilia and elevated serum immunoglobulin E. Most cases have been described predominantly in Chinese and Japanese people. KD has been confused with angiolymphoid hyperplasia with eosinophilia, from which it probably should be distinguished as a separate entity. The course is usually benign except for the potential cosmetic disfigurement. The diagnosis may be suggested by a fine-needle aspirate but is established by a biopsy. A case of KD in a 42-year-old male with recurrent left submandibular swelling is presented together with a brief review of the literature.

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Clinical and histopathologic differences

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon lesion with a predilection for skin of the head and neck region. It usually presents as one or a few smooth-surfaced nodules which are often asymptomatic. Histologically the nodules reveal two components: abnormal proliferating vessels and a cellular infiltrate consisting of lymphocytes and eosinophils, involving both the dermis and the subcutaneous tissue. A case is presented of an unusually large recurrent lesion on the scalp.

The relationship between the disorder known in Japanese and Chinese literature as Kimura's disease and that known in Western literature as angiolymphoid hyperplasia with eosinophilia (ALHE) has been the subject of debate. Many reports have used the terms synonymously. We have reviewed the histological and clinical features of 4 cases, all occurring in Caucasians, 2 of which are typical of Kimura's disease and 2 of ALHE. Analysis of the cases indicates that the histological features of the 2 disorders are sufficiently different to warrant their recognition as 2 distinct entities. The histological and clinical features of Kimura's disease are most consistent with an allergic or autoimmune process in which blood vessels, lymphocytes and eosinophils participate. Those of ALHE suggest a primary, probably neoplastic disorder of vascular endothelium with a variable and secondary inflammatory response. Although there is some clinical overlap between patients with the 2 disorders, the histological features are distinctive, and the 2 terms should not be used synonymously.

Although Kimura's disease has often been considered to be identical to angiolymphoid hyperplasia with eosinophilia (epithelioid haemangioma), recent studies suggest that they are different clinicopathological entities. In this study, we have made a detailed morphological comparison of 10 cases of epithelioid haemangioma and 40 cases of Kimura's disease occurring in the Chinese population. The epithelioid haemangiomas occurred in the subcutaneous tissue, skin and maxillary antrum, whereas Kimura's disease affected the subcutaneous tissue, major salivary glands and lymph nodes. Distinctive features of epithelioid haemangiomas were exuberant proliferation of vessels lined by cuboidal to hobnail endothelial cells with irregular nuclei and cytoplasmic vacuoles, fibromyxoid matrix, involvement of muscular coat of blood vessels and zonation of inflammatory infiltrate towards the peripheral portion of the lesion. Distinctive features of Kimura's disease were florid lymphoid infiltrate with prominent lymphoid follicles, vascularization of germinal centres, germinal centre necrosis, marked eosinophilia with or without eosinophil abscess formation, proliferation of high endothelial venules, and sclerosis. The histological features suggest that epithelioid haemangioma is a proliferation of atypical endothelial cells, possibly neoplastic, that is associated with a variable inflammatory infiltrate, whereas Kimura's disease is primarily an inflammatory condition in which high endothelial venules are usually found.

We report an interesting case of Kimura’s disease (KD) that presented as a giant head and neck mass. KD is a rare chronic inflammatory disorder and usually presents as small subcutaneous swellings in the head and neck region. Such a large mass of 15 × 17.5 cm as seen in our patient has previously not been reported, to the best of our knowledge. The radiological picture was that of an enlarged subcutaneous mass with heterogeneous enhancement of the salivary gland. The histological findings were helpful in identifying the lesion as KD. The combination of these two aided us in differentiating it from other lesions such as follicular lymphoma, angiolymphoid hyperplasia with eosinophilia (ALHE) and Langerhan’s histiocytosis. There is no standard protocol for management of this disease owing to its rarity. Due to the extensive and ill-defined nature of the lesion in our patient, we have started the patient on systemic high-dose corticosteroid therapy to downsize the lesion and we plan to follow up with surgical excision if needed.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign reactive vasoproliferative disease, presenting as painless vascular papules or nodules in the dermal and subcutaneous tissue particularly in the head and neck region. The etiology is uncertain whether; reactive or neoplastic; but its association with Tuberculosis is rare and very few cases have been reported so far. We present a nine year old girl with a swelling on the dorsum of foot since 3 months. She reported to have taken treatment for Tuberculosis when she was six years. Excisional biopsy showed vascular proliferation with prominent mixed inflammatory component, diagnosed as ALHE based on histopathological and Immunohistochemical methods. Its association in the present case showed possibly a result of complex immunological mechanism secondary to chronic infection like tuberculosis. We present a case of ALHE with a very rare presentation on the dorsum of the foot in a 9 year old female with a history of Tuberculosis.

Sir, Angiolymphoid hyperplasia with eosinophilia (ALHE) is an unusual condition that usually manifests in young to middle‐aged caucasian adults as a single pinkish‐brown nodule, several centimetres in size, preferentially at the retroauricular area or as a group of smaller nodules distributed on the head and neck.1 In addition to tissue eosinophilia, some patients may develop peripheral blood eosinophilia and markedly elevated serum IgE levels, features typically seen in Kimura's disease.1 Treatment of ALHE is problematic, as the cause is unknown. Excision may be curative, but is difficult to perform in extensive cases.2 Alternative options including systemic corticosteroids, radiation, laser surgery, cryosurgery, cytotoxic agents and pentoxifylline have been tried with variable responses and relapses after withdrawal.3 As interleukin (IL)‐5, the major cytokine involved in production, mobilization and activation of eosinophils, has been found to be elevated in patients with ALHE,4, 5 blocking the interaction between IL‐5 and its receptor might be a reasonable therapeutic approach.

Selection de resumes interessant les acquisitions nouvelles en pathologie tumorale des tissus mous au cours des annees 88 et 89: description de 2 nouvelles entites. La tumeur fibrohistiocytaire plexiforme et le myofibroblastome palissadique, distinction entre haemangiome epithelioide et maladie de KIMURA et interet de l'anticorps anti EMA comme outil diagnostique courant dans le cadre des tumeurs des tissus mous

A 40‐year‐old white Caucasian man presented with a 6‐month history of a 1.5 × 1.5 cm nodular lesion on his left arm. No history of trauma preceeded the lesion and the patient was in good general health.Dermatologic examination revealed a solitary, red, painless nodule located on the left arm (Fig. 1). Histopathologic examination of punch biopsy revealed lymphoid follicles, some of which had active germinal centers in the deep dermis and subcutaneous tissue. Mixed infiltration of lymphocytes, plasma cells and many eosinophils were present in the interfollicular region. Infiltration of the germinal centers by eosinophils, causing partial destruction, was observed (Fig. 2A). There was also proliferation of thin‐walled small vessels and fibrosis in some parts of the interfollicular areas (Fig. 2B). Although Kimura's disease (KD) and angiolymphoid hyperplasia with eosinophilia (ALHE) were considered in the differential diagnosis, it was diagnosed as KD based on these histologic features. Further laboratory examinations were performed. Serum IgE was normal and peripheral eosinophilia was absent.The lesion regressed after biopsy. Complete healing was achieved with intralesional corticosteroid (triamcinolone acetonide) treatment.

Angiolymphoid hyperplasia with eosinophilia (AHE) was first described by Wells and Whimster in 1969 (1). These authors considered the process to be a late stage of Kimura’s disease, a disorder described in the Japanese literature 20 years earlier (2). Following this description, the concept that AHE and Kimura’s disease were the same entity was widely accepted. It later became clear that the entities are different, and currently most authors believe that AHE and Kimura’s disease are two separate entities (3–8). To complicate the issue further, different names have been used to describe AHE, to wit: atypical pyogenic granuloma (9), pseudopyogenic granuloma (10), inflammatory angiomatous nodule (10), papular angioplasia (11), inflammatory arteriovenous hemangioma (12), intravenous atypical vascular proliferation (13), cutaneous histiocytoid hemangioma (7,14), and epithelioid hemangioma (15). Many of these terms are confusing and in our opinion it is best not to apply them to this particular entity. Take for example the term histiocytoid hemangioma (14). This term has been used to describe a wide spectrum of vascular proliferations, both benign and malignant, all of which are histopathologically characterized by the presence of endothelial cells with a histiocytoid appearance. Because this term is not specific and does not designate a single entity it is better not to use it. Another confusing term is epithelioid hemangioma (15), since it can be confused with epithelioid hemangioendothelioma, an entirely different clinicopathologic entity.

Angiolymphoid hyperplasia with eosinophilia and vascular tumors of the head and neck