Kimura disease of the eyelid in an Indian man

© 2014 The Authors. doi: 10.2340/00015555-1623 Journal Compilation © 2014 Acta Dermato-Venereologica. ISSN 0001-5555 Kimura’s disease (KD) is a chronic inflammatory disease characterized by subcutaneous nodular lesions in the head and neck area, eosinophilia, an elevated serum level of IgE and, histologically, the formation of lymphoid follicles with massive eosinophilic infiltration (1). There are several case reports of patients with KD with prurigolike skin lesions (2, 3). We report here a case of KD that presented with subcutaneous nodules of the eyelids and prurigo-like eruption on the trunk and extremities. Serum concentrations of thymus and activation-regulated chemokine (TARC/CCL17), eotaxin-3/CCL26 and vascular endothelial growth factor (VEGF) were measured before and after treatment.

Much controversy has existed with regard to the relationship between Kimura's disease and angiolymphoid hyperplasia with eosinophilia. They were initially thought to represent the same disease spectrum, but it has now been widely accepted that they are two separate disease entities. To our knowledge, there have been no reports to date describing a case of both Kimura's disease and angiolymphoid hyperplasia with eosinophilia coexisting in the same patient. We describe a patient presenting initially with a right postauricular subcutaneous swelling and subsequently developing multiple erythematous facial papules and nodules. The medical literature is reviewed. Clinical features of the right postauricular subcutaneous swelling and multiple erythematous facial papules/nodules suggest Kimura's disease in the former and angiolymphoid hyperplasia with eosinophilia in the latter. Histopathological examinations of these lesions helped to confirm the diagnosis of Kimura's disease and angiolymphoid hyperplasia with eosinophilia, respectively. Kimura's disease and angiolymphoid hyperplasia with eosinophilia can coexist in the same patient. Coexistence of the two types of lesions in one patient may also be considered evidence that Kimura's disease and angiolymphoid hyperplasia with eosinophilia form a spectrum in one disease.

Kimura's Disease

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

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign clinical entity characterized by the presence of a variable number of papules, plaques or nodules of the dermis and subcutaneous tissues. ALHE shows a predilection for the head and neck area. Orbital involvement is unusual. A 20-year-old male presented with a 4-month history of swelling of the upper lid of right eye. Magnetic resonance imaging orbit revealed altered signal density seen in supero-medial aspect of right eyeball with adjacent inflammatory changes. The patient underwent anterior orbitotomy and excision biopsy of the mass under general anesthesia. Histopathology of the mass showed vascular proliferation and chronic inflammation accompanied by infiltration of eosinophils, which confirmed the diagnosis of ALHE. The patient recovered both functionally and cosmetically and had no signs of recurrence after 6 months. Angiolymphoid hyperplasia with eosinophilia with orbital involvement in males is a rare clinical entity, and further work is required to accurately describe its incidence, etiology and presentation. ALHE can be diagnosed and differentiated from Kimura's disease (KD) on histopathological grounds. The presence of vascular hyperplasia with plump of endothelial cells protruding into the lumen is the most important discriminator in establishing the diagnosis of ALHE. Such distinction is crucial for the patient because ALHE is not associated with any of the systemic manifestations present in KD.

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, localised, chronic inflammatory disease. This benign disease involves subcutaneous tissues, the major salivary gland, and lymph nodes primarily in the head and neck area. Clinical details and stained slides of all cases reported as Kimura's disease over a 10-year period were reviewed. There were eight cases of Kimura's disease. The mean age of patients was 22.8 years. One case showed associated nephrotic syndrome and two cases were associated with peripheral blood eosinophilia. All cases showed the typical histopathological features of Kimura's disease. Kimura's disease was first reported in China in 1937. The cause of Kimura's disease is unknown and many theories have been proposed. The eight cases reported here illustrate some of the variations in the mode of presentation and in the histological features of Kimura's disease. Kimura's disease should be considered in the differential diagnosis of patients who present with head and neck swellings and lymphadenopathy, and investigated accordingly.

Angiolymphoid hyperplasia with eosinophilia versus Kimura's disease:a case report and a clinical and histopathological comparison*

Recently, human herpesvirus 8 (HHV-8) has been isolated from almost all cases of Kaposi's sarcoma. It has not been found in most cutaneous hemangioproliferative disorders other than Kaposi's sarcoma. Benign vascular lesions including Kimura's disease were not found to contain the HHV-8 DNA sequence. However, there has been contradictory data concerning the presence of HHV-8 in angiolymphoid hyperplasia with eosinophilia (ALHE). Clonality studies in ALHE and Kimura's disease were rare. We performed polymerase chain reaction (PCR)-based analysis to determine whether HHV-8 is present and heteroduplex analysis of rearranged T-cell receptor (TCR) gene for clonality assessment in paraffin-embedded skin biopsy samples of 7 ALHE and 2 Kimura's disease, taken from immunocompetent patients. HHV-8 could not be identified in all the cases of ALHE and Kimura's disease. Although 2 cases (2/7) of ALHE and 2 cases (2/2) of Kimura's disease showed positive result for PCR analysis of TCR, all the cases were negative for heteroduplex-PCR. We suggest that HHV-8 may not involve in a pathogenetic role in ALHE and Kimura's disease and the failure to demonstrate clonality may be consistent with the reactive nature of these diseases and lack of malignant transformation. In addition, heteroduplex-PCR can be applied to confirm doubtful cases of lymphoma in that heteroduplex-PCR is more specific than PCR as seen in our study.

Kimura's disease in a 50-year-old Tunisian man

Kimura's disease is a benign chronic inflammatory disease, common in Asian males and rare in Western people. Clinically, Kimura's disease is characterized by subcutaneous nodular lesions, usually localised in head and neck, often associated with regional lymphadenopathy. Peripheral blood eosinophilia and elevated serum IgE are often observed. We report a case of a 40-year-old Italian patient presenting with nodular subcutaneous lesions and peripheral eosinophilia. Based on clinical, histopathological and laboratory findings, a diagnosis of Kimura's disease was made. The patient was treated with very low doses of cyclosporine A with no evidence of disease recurrence over the following 8 years. However, the discontinuation of cyclosporine A determined a relapse of the disease. The relevance of this case is due to the rarity of the disease in Italy, to its peculiar clinical presentation and, moreover, it is the first case in literature that has a good response to treatment with low doses of cyclosporine A, documented in an 8-year follow-up.

The clinicopathologic features of nine patients with Kimura's disease and 15 patients with angiolymphoid hyperplasia with eosinophilia (ALHE) were studied and compared in order to clarify the confusion between these two entities. The common features shared by both conditions included male predominance, predilection for the head and neck regions, tendency to recur, and vascular nature of the lesion with lymphoid and eosinophilic infiltrates. However, Kimura's disease was usually seen in younger individuals for a longer duration and occurred as a deeply seated, large soft-tissue mass, without significant change of the overlying skin initially. In addition, it was often accompanied by peripheral blood eosinophilia and elevated serum IgE. In contrast, ALHE lesions were multiple small dermal papular or nodular eruptions observed in older patients and present for a shorter duration; they were less frequently accompanied by peripheral blood eosinophilia. The main histopathological difference was the presence of "histiocytoid" or "epithelioid" blood vessels in ALHE but not in Kimura's disease. Kimura's disease was further characterized by eosinophilic folliculolysis; IgE deposits in the germinal centers; and frequent involvement of regional lymph nodes, salivary glands, and skeletal muscles. The eosinophilic infiltration, especially the formation of eosinophilic microabscesses, along with increased number of small blood vessels, perinodal eosinophilic infiltration, and eosinophilic folliculolysis characterized the nodal involvement by Kimura's disease. Our study indicates that Kimura's disease and ALHE are two distinct clinicopathologic entities. We place particular emphasis on the involvement of regional lymph nodes in Kimura's disease. In addition, we observed Charcot-Leyden crystals and polykaryocytes in both conditions. One of the patients with Kimura's disease also had an associated nephrotic syndrome.

The analysis of published clinical observations on the diagnosis and treatment of patients with Kimura's disease, which is a rather rare chronic inflammatory disease of human soft tissues of unknown etiology, is presented. The onset of Kimura's illness is hardly noticeable. The disease proceeds sluggishly, with a varied clinical picture, most often with the formation of single or multiple subcutaneous nodules or tubercles and swelling in the head and neck on one or both sides. Other localizations of lesions are also described: limbs, orbit, eyelids, palate, pharynx, armpit, chest, inguinal region. The affected areas gradually increase in size over many months or years. Other clinical manifestations of Kimura's disease are presented in the form of edema of the extraocular muscle, juvenile temporal arteritis, sleep apnea in cases of damage to the larynx, and life-threatening coronary artery spasm in children. There are reports of other rare systemic clinical signs of Kimura's disease in the form of pruritus, eczema and rash. The disease is often associated with regional lymphadenopathy and/or salivary gland involvement, eosinophilia, and an increase in serum immunoglobulin E from 800 to 35,000 IU/mL. The disease may be accompanied by kidney damage in the form of glomerulonephritis and nephrotic syndrome. Most reported cases of Kimura's disease occur in young Asian men in their 20s and 30s, sporadically the disease is diagnosed in Europeans, North Americans, Caucasians, and Africans. Diagnosis of Kimura's disease is often clinically difficult. It is primarily based on excisional biopsy of the lesion or regional lymph nodes, and the histopathological findings are consistent regardless of the location of the lesion. Morphologically, Kimura's disease is characterized by the presence in biopsy specimens of such histological features as reactive follicular hyperplasia with prominent follicles surrounded by fibrous tissue. Interfollicular infiltrates are rich in plasma cells and eosinophils, which form diffuse eosinophilic microabscesses associated with granulomatous inflammation. The vessels are numerous, with an increase in postcapillary venules. At the same time, the blood vessels remain thin-walled, with cuboidal endothelial cells. Treatment issues are highlighted, and differences between Kimura's disease and angiolymphoid hyperplasia with eosinophilia are presented.

BACKGROUNDKimura’s disease is an inflammatory disease that is usually found in the deep lymph nodes of the head and neck. While rare, It is most frequently seen in young men. The oral cavity and salivary glands may also be involved. There are no reports on tumor occurring in soft palate. We have encountered a case of Kimura’s disease in the soft palate of an elderly woman.CASE SUMMARYA 63-year-old elderly Chinese woman with a slowly growing mass in the upper jaw was referred to our service. A biopsy to the mass was taken after the patient was referred to our service. The tumor was diagnosed as benign. We performed cervical lymph node puncture and partial surgical excision of the lesion. The tumor, which showed signs of marked follicular hyperplasia with follicles surrounded by eosinophils and lymphocytes, was located within the soft palate. Kimura’s disease was diagnosed after histopathologic examination of the resected tissue. The etiology of Kimura’s disease is not fully understood. One current model includes T-cells involvement with cytokines also playing a role. The patient was without evidence for recurrence of partially resected area 6 mo later. This report shows that Kimura’s disease is not limited to the head, neck, and salivary gland lymph nodes. We present a case of a tumor in soft palate. This location adds another possible site for consideration during the differential diagnoses of a slowly growing mass.CONCLUSIONThe present case illustrates a characteristic description of Kimura’s disease. This case highlights the main differences between Kimura’s disease and angiolymphoid hyperplasia with eosinophilia.

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