KIMURA'S disease – An E[X]clusive condition

Abstract

Kimura's disease is a rare autoimmune disease of unrecognized etiology. Due to its unspecific clinical presentation and laboratory studies, Kimura's is a diagnosis of exclusion . A systematic multidisciplinary approach is mandatory to rule out the other common causes of cervicofacial lymphadenopathy. A thorough Histopathological examination including immunohistochemical analysis along with the presence of specific biochemical markers, including raised Absolute eosinophilic count is necessary to conclude the diagnosis as Kimura's Disease. In this article we present a case of a middle aged Asian woman with cervicofacial lymphadenopathy with no associated illness. The above described protocol of clinical, radiological and histolopathological investigations was followed before establishing the final diagnosis of Kimura's. The review of literature on contemporary management and prognosis is discussed.

Kimura Disease of the Thigh Treated With Surgical Excision and Dupilumab

“…3,6 Kimura disease affects mostly Asian males with a median age of 32 years, and male-to-female ratio is about 5:3. 3,6 Various symptoms may occur in KD patients. In a review of 22 patients of KD, Kottler et al 7 indicated a history of pruritus or atopic diathesis in 40% of patients, whereas 58% of patients had atopic dermatitis or lichen simplex chronicus.…”

“…Allergic reaction, infection, and autoimmune reactions may play a role in the pathogenesis. 6 Eosinophilia (up to 10%-20% eosinophils and increased leucocytes) and increased serum IgE levels are characteristic. 9 Elevated IgE, tumor necrosis factor-α, interleukin (IL)-4, IL-5, IL-13, and mast cell levels are noted in blood and lesion tissue.…”

“…[3][4][5] Current treatments for KD include surgical excision, steroid medication, and radiation therapy; however, optimal therapy is not known. 3,6 Here we report a 36-year-old Asian man with an enlarging mass in the left thigh and chronic eczema of the abdomen and lower legs. Kimura disease was diagnosed after surgical excision.…”

Case Report: A Rare Case of Nasal Forehead Mass in Kimura's Disease

“…Conspicuous features of Kimura's disease on histopathology include hyperplastic lymphoid follicles with prominent germinal centers and eosinophilic infiltration. Laboratory tests show that eosinophils on routine blood testing and IgE in serum are significantly increased in patients with Kimura's disease (6)(7)(8)(9)(10)(11). Sometimes, Kimura's disease can occur in other organs, especially the kidneys, where it takes the form of membranoproliferative glomerulonephritis (12,13).…”

Kimura’s disease as an uncommon cause of post auricular swelling

“…The histological picture with the formation of lymphoid follicles and intense aggregates of eosinophils (which sometimes form micro abscesses), vascular proliferation, and fibrosis are highly suggestive of KD. 12,13 Differential diagnosis of KD would include angiolymphoid hyperplasia with eosinophilia (ALHE), Kikuchi disease, Mikulicz's disease, and most importantly Hodgkin and non-Hodgkin lymphoma.…”