Kimura disease of extremity: Unusual manifestation in a long bone

An Unusual Clinical Presentation of Kimura's Disease Occurring on the Buttock of a Five-year-old Boy

“…The disease is most prevalent in Asians, uncommon in Caucasians, and rare in Africans. It has been suggested that one common factor is Asian ancestry 3 . To date, the etiology of KD remains unclear, though various mechanisms have been proposed.…”

“…Although the disease can manifest at any age, most cases have been reported in the second and third decades of life 2,3 . An earlier review of 194 cases in the Japanese literature revealed that slightly over one-third of the cases occurred in the second decade 7 .…”

“…It is characterized by painless subcutaneous masses, blood and tissue eosinophilia, and markedly elevated serum IgE levels. Although the disease can present at any age, most cases have been reported in the second or third decades of life 2,3 . Clinically, the subcutaneous soft tissue masses occur predominantly in the head or neck.…”

Orthopaedic · Radiology · Pathology Conference: Hand Mass in an 18-year-old Woman

“…Although both have blood vessels lined by endothelial cells with perivascular infiltration of eosinophils, ALHE is unique in that its endothelial cells are characteristically plump; the endothelial cells in Kimura's disease are much flatter. A second key difference between ALHE and Kimura's disease is that the latter typically affects deeper tissues, such as lymph nodes and salivary glands, than the former [10].…”

Thrombotic storm in Kimura disease

“…Kimura disease is a rare chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region with regional lymphadenopathy or salivary gland enlargement. Other sites of involvement including limbs [11,12], axilla, groin, trunk [13], oral cavity, and colon [14] have been reported. Kidney involvement resulting in nephrotic syndrome has been reported [15,16].…”