Kimura's disease

Kimura's disease is a benign rare chronic inflammatory disorder. Its primary symptoms are subdermal lesions in the head or neck or painless unilateral inflammation of cervical lymph nodes. Kimura's disease is a benign rare chronic inflammatory disorder. Its primary symptoms are subdermal lesions in the head or neck or painless unilateral inflammation of cervical lymph nodes. Its cause remains unknown. Reasons such as an allergic reaction, tetanus toxoid vaccination, or an alteration of immune regulation are suspected. Other theories like persistent antigenic stimulation following arthropod bites and parasitic or candidal infection have also been proposed. To date, none of these theories has been substantiated. The pathophysiology of Kimura's disease remains unknown, although an allergic reaction, trauma, and an autoimmune process have all been implicated as possible causes. The disease is manifested by an abnormal proliferation of lymphoid follicles and vascular endothelium. Peripheral eosinophilia and the presence of eosinophils in the inflammatory infiltrate suggest it may be a hypersensitivity reaction. Some evidence has indicated TH2 lymphocytes may also play a role, but further investigation is needed. Kimura's disease is generally limited to the skin, lymph nodes, and salivary glands, but patients with Kimura's disease and nephrotic syndrome have been reported. The basis of this possible association is unclear. An open biopsy is the chief means by which this disease is diagnosed. 'Lymphoid nodules with discrete germinal centers can occupy an area extending from the reticular dermis to the fascia and muscle. Follicular hyperplasia, marked eosinophilic infiltrate and eosinophilic abscesses, and the proliferation of postcapillary venules are characteristic histological findings. Centrally, thick-walled vessels are present with hobnail endothelial cells. Immunohistochemical evaluation of the lymphoid nodules demonstrates a polymorphous infiltrate without clonality. Reports have also demonstrated the presence of plasmacytoid dendritic cells in a lesion of Kimura disease. Histopathological examination is an effective way to establish the diagnosis.'