Kimura disease is most common in Asia. Patients with Kimura disease are usually young adults, although the disease may present during adolescence, particularly in Chinese and Japanese males. Men are affected more often than women. About 15%-20% of patients with Kimura disease also have nephrotic syndrome, although the pathophysiologic basis for this association is unclear, and acute glomerulonephritis is seen in about one-half of patients.
It is thought that the disease may represent a hypersensitivity or autoimmune process, but regardless, the end result is clearly an abnormal proliferation of lymphoid follicles and vascular endothelium. Of note, angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura disease, once thought to be disease variants, are now recognized to be separate, distinct diseases with specific clinical and histologic features.
Pediatric Patient Considerations:
Rare in children.
L92.8 – Other granulomatous disorders of the skin and subcutaneous tissue
399894006 – Kimura disease
Differential Diagnosis & Pitfalls
- Reactive lymphadenopathy – Generally less prominent and more "shotty," and is without the peripheral eosinophilia and elevated IgE.
- Lymphoma – The lymphadenopathy is typically more generalized and would not necessarily involve only the head / neck. Generally, there is other evidence of a lymphoproliferative disorder including type B symptoms (eg, fever, night sweats, weight loss).
- Kikuchi-Fujimoto disease
- Rosai-Dorfman disease
- Angiolymphoid hyperplasia with eosinophilia (ALHE)