Kimura's disease
| Kimura disease | |
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| Micrograph of Kimura disease. H&E stain. | |
| Classification and external resources | |
| DiseasesDB | 31710 |
| eMedicine | article/1098777 |
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.
The first known report of Kimura's disease was from China in 1937, when Kimm and Szeto identified seven cases of the condition. 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."
Its cause 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 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 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 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.
Kimura's disease is said to be predominantly seen in males of Asian descent. However, a study of 21 cases in the United States showed no racial preference, of 18 males and three females (male/female ratio 6:1), 8 to 64 years of age (mean, 32 years), there were seven Caucasians, six Blacks, six Asians, one Hispanic, and an Arab.
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's disease, but recurrence of the lesions have been observed once this therapy is stopped.
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