Kimura Disease

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اشترك في: الخميس إبريل 04, 2013 10:28 pm

Kimura Disease

مشاركةبواسطة دكتور كمال سيد » الأحد مارس 09, 2014 5:53 pm

Kimura Disease

(painless subcutaneous nodules or plaques)

Kimura's disease is a rare, benign chronic inflammatory condition which commonly occurs among the Oriental population.

The exact cause of this disease is unknown but it is believed to result from an immunological reaction that is allergic or autoimmune in nature.

The lesion shows no evidence of malignant transformation. Recurrence may occur following surgical excision.

Although the Asians are commonly affected some cases have been reported in the Caucasians and in the African population.

Age: Usually occurs in the second and third decade of life.

Site: Commonly located in the head and neck region particularly in the pre or post auricular region and rarely in the oral mucosa, orbit, and the scalp.

Rarely, Kimura's disease may occur in the parotid or other salivary glands.

Other rare sites include limbs and trunk, vulva, spermatid cord, inguinal and axillary lymph nodes and peripheral nerves.

Clinical presentation: The patient presents with painless subcutaneous nodules or plaques.

Lymphadenopathy is present in more than 50% cases.

Kimura's disease is often associated with peripheral blood eosinophilia, raised ESR and serum IgE.

Accurate diagnosis of Kimura's disease is based on clinical and histological findings.

Microscopic features:

Features of the skin lesion :

Lymphoid follicles with prominent germinal centre.

Fibrous tissue surrounding the lymphoid follicles.2

Inflammatory cellular infiltrate in the interfollicular area consisting of small lymphocytes, plasma cells andeosinophils. In areas, scattered eosinophilic microabscesses are identified.

Thin-walled blood vessels are increased in number lined by flat or cuboidal endothelial cells.

Features of the involved lymph node:

. Marked hyperplasia of the germinal centre. Germinal centres are often well vascularized and containeosinophilic deposits and polykaryocytes (Warthin-Finkeldey type).

Extensive eosinophilic infiltration consisting of mature eosinophils in follicular and interfollicular areas andoccasional eosinophilic microabscess formation.

. Proliferation of thin-walled blood vessels in the paracortex.

. Increase in the number of plasma cells and mast cells in the paracortex.4

Differential diagnosis:

Angiolymphoid hyperplasia with eosinophilia :

Previously, angiolymphoid hyperplasia with eosinophilia and Kimura's disease were regarded as the same lesion. But these conditions are now regarded as two separate entities.

Multiple, small well circumscribed dermal or subcutaneous papules or nodules with well-defined borders.
(In Kimura's the lesion is located in the deep soft tissue or the subcutis without significant change of the overlying skin initially ).

. Less frequently associated with lymphadenopathy and peripheral blood eosinophilia.

. Histologically, unlike Kimura's disease the blood vessels are lined by plump epithelioid like endothelial cells. These cells demonstrate large nucleus and abundant eosinophilic cytoplasm. Prominent cytoplasmic vacuoles are present.

Dense lymphocyic infiltration is present in only some cases of ALHE.

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اشترك في: الخميس إبريل 04, 2013 10:28 pm

Re: Kimura Disease

مشاركةبواسطة دكتور كمال سيد » الأحد مارس 09, 2014 5:56 pm


Kimura disease is a chronic inflammatory disorder of unknown etiology that most commonly presents as painless lymphadenopathy or subcutaneous masses in the head or neck region. The first report of Kimura disease was from China in 1937, in which Kimm and Szeto[1] described 7 cases of a condition they termed "eosinophilic hyperplastic lymphogranuloma." The disorder received its current name in 1948, when Kimura et al[2] noted the vascular component and referred to it as an "unusual granulation combined with hyperplastic changes in lymphoid tissue."

Controversy has existed in the literature regarding whether Kimura disease and angiolymphoid hyperplasia with eosinophilia (ALHE) are the same entity. Some authors believe that Kimura disease represents a chronic, deeper form of ALHE; however, most recent papers distinguish the 2 on the basis of clinical and histopathologic characteristics.[3] ALHE appears to represent an arteriovenous malformation with secondary inflammation. Kimura disease may represent a primary inflammatory process with secondary vascular proliferation.

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