Kimura's disease

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.

Kimura disease is a benign rare chronic inflammatory disorder of unknown etiology that involves the lymph nodes and subcutaneous tissue of the head and neck region. Most cases have been described predominantly in middle aged Asian people. The lesion is benign, but it may easily be mistaken for a malignant tumor. Kimura disease has been confused with angiolymphoid hyperplasia with eosinophilia (ALHE), from which it probably should be distinguished as a separate entity. The diagnosis may be suggested by a fine needle aspirate, but is established by a biopsy. The treatment of choice is surgical removal. We hereby, present a case of Kimura disease in 18 year old male presented with multiple swellings over bilateral retro-auricular and occipital region.DOI:10.21276/APALM.1695

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

Kimura's disease (KD) is an immune-mediated disorder which mainly affects Asian men. It appears as head and neck subcutaneous masses, with inflammatory infiltrate and elevated serum immunoglobulin E levels. The clinical presentation of KD resembles that of various diseases. Here, we report the case of a 30-year-old Filipino man with KD mimicking cavernous hemangioma who was treated by surgery. Careful survey for possible KD cases is crucial. Misdiagnoses are prone to futile interventions and unwanted effects. Surgery with adjuvant therapy is superior to other forms of KD treatment.

Kimura's disease is a rare chronic inflammatory disease of unknown etiology, presenting as painless subcutaneous nodules with lymphadenopathy and peripheral eosinophilia, mainly disturbing the head and neck region. It mainly affects Asian males in their 2(nd) to 4(th) decade of life. One such case of Kimura's disease, which is uncommon in Indian natives, is reported. A male patient presented with an insidious onset of a progressive painless disfiguring swelling over his nose since four years, which was associated with nasal obstruction and post aural swelling with a history of an inconclusive incisional biopsy. Clinical examination showed a bilobed subcutaneous swelling present over the nose and a collapsed nasal valve area on anterior rhinoscopy. FNAC was non-diagnostic and CT scan showed a mildly enhancing mass lesion over the external nose. Complete surgical excision was performed. Diagnosis was confirmed upon postoperative histopathology. During his 2nd week follow up, the patient had a small nasal recurrence, which was treated medically with oral steroids, cetirizine, and pentoxyphylline for 4 weeks. The patient was disease free for 6 months. Kimura's disease, although difficult to diagnosis clinically, should be considered in the differential diagnosis of patients who have a primary lymphadenopathy with eosinophilia with or without subcutaneous nodules. It should be investigated accordingly as the disease has an indolent course and good prognosis.

Introduction: Kimura disease is a rare chronic inflammatory disorder of unknown etiology predominantly affecting young Asian males, characterized by painless subcutaneous masses in the head and neck region, peripheral eosinophilia, and elevated serum immunoglobulin E levels. This condition remains underreported in Southeast Asian countries, particularly Indonesia, leading to significant diagnostic delays. Case presentation: We present the case of a 48-year-old Indonesian male from Bali who presented with a retroauricular mass persisting for 10 years prior to definitive diagnosis. The patient had undergone surgical excision one month prior and was subsequently referred for further evaluation. Laboratory investigations demonstrated leukocytosis with a white blood cell count of 13.82 × 103/µL, peripheral eosinophilia of 8.60%, absolute eosinophil count of 1.19 × 103/µL, and elevated total serum immunoglobulin E of 168.16 KUI/L. Renal function was preserved without proteinuria despite prolonged disease duration. Histopathological examination confirmed Kimura lymphadenitis with reactive follicular hyperplasia, dense eosinophilic microabscesses, vascular hyalinization, and multinucleated Warthin-Finkeldey giant cells. The patient was managed with surgical excision followed by oral methylprednisolone 8 mg daily with planned tapering. Conclusion: This case highlights diagnostic challenges of Kimura disease in Indonesian clinical settings and the importance of histopathological confirmation.

Kimura’s Disease of the Parotid Region: Report of 2 Cases and Review of the Literature

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

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 (KD) is an allergic, inflammatory disorder of unknown cause. The typical presentation is in a young Oriental male with nontender subcutaneous swellings in the head and neck region, lymphadenopathy, peripheral eosinophilia, and elevated serum IgE. Many patients with KD also develop renal involvement. Treatment options range from conservative observation for asymptomatic patients to surgical excision, steroid therapy, and radiotherapy for symptomatic patients. Two cases of KD are presented. These emphasize not only the variability of disease presentation, but also the pitfalls of therapy leading to frequent recurrences. The literature is reviewed, summarizing patient presentation, differential diagnosis, current theories on causation, and therapy. A treatment protocol is also proposed.

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

Kimura's disease is a chronic inflammatory disorder, while cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral small vessel disease. A 56-year-old Chinese male with a 6-year history of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy was admitted due to recurrent cerebral infarcts. He also had a concurrent diagnosis of Kimura's disease accompanied by hypereosinophilia and elevated serum immunoglobulin E levels. Although the patient had been on oral antiplatelet therapy, no preventive treatment had been previously administered for the hypereosinophilia or high immunoglobulin E levels. We conducted follow-up to track disease progression and to review the management approach, including its strengths and shortcomings. This case represents a rarely reported instance of Kimura's disease coexisting with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. It underscores the importance of differentiating stroke etiologies and illustrates the potential for complications when hypereosinophilic conditions are left untreated.

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.

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.

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.