Kimura 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.[1]
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History
The first known report of Kimura disease was from China in 1937, when Kimm and Szeto identified seven cases of the condition.[2] It first received its name in 1948 when Kimura and others noted a change in the surrounding blood vessels and referred to it as "unusual granulation combined with hyperplastic changes in lymphoid tissue."[3]
Cause
The cause of Kimura disease remains unknown. Reasons like an allergic reaction 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 have been substantiated.[1]
Pathophysiology
The pathophysiology of Kimura Disease remains unknown, although an allergic reaction, trauma, and an autoimmune process have all been implicated as the possible cause. 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 that Kimura Disease may be a hypersensitivity reaction. Some evidence has indicated that TH2 lymphocytes may also play a role, but further investigation is needed.
Kimura Disease is generally limited to the skin, lymph nodes, and salivary glands, but patients with Kimura Disease and nephrotic syndrome have been reported. The basis of this possible association is unclear.[1]
Frequency and Distribution
Kimura Disease is predominantly seen in males of Asian descent. The disease has rarely been reported in the United States. On an international level, the prevalence of the disease is not known.[1]
Treatment
Observation is acceptable if the lesions are neither symptomatic nor disfiguring. Intralesional or oral steroids can shrink the nodules but seldom result in cure.
Cyclosporine has been reported to induce remission in patients with Kimura Disease. However, recurrence of the lesions have been observed once this therapy is stopped.
Radiotherapy has been used to treat recurrent or persistent lesions. However, considering the benign nature of this disease, radiation should be considered only in cases of recurrent, disfiguring lesions.
Surgery has been considered the mainstay of therapy. However, recurrence after surgery is common.[4]
References
- ^ a b c d Sena Lee (May 2007). "Kimura Disease". eMedicine. http://www.emedicine.com/derm/topic212.htm. Retrieved 2008-07-20.
- ^ Kimm HT, Szeto C (1937). "Eosinophilic hyperplastic lymphogranuloma, comparison with Mikulicz's disease". Proc Chin Med Soc: p. 329.
- ^ Kimura T, Yoshimura S, Ishikawa E (1948). "On the unusual granulation combined with hyperplastic changes of lymphatic tissues". Trans Soc Pathol Jpn: pp. 179–180.
- ^ http://emedicine.medscape.com/article/1098777-treatment
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