A 52-year-old Asian man with a history of bilateral submandibular gland and cervical lymph node enlargement underwent a biopsy of his right submandibular gland. The resultant specimen is shown in the images below.
There is a dense inflammatory infiltrate consisting predominantly of eosinophils, lymphocytes and plasma cells. Lymphoid germinal centers are prominent. An area of eosinophilic abscess formation is present in the upper center of the first image. In the background, there is a pronounced proliferation of post-capillary venules, many of which show marked peri-venular fibrosis. Peripheral blood eosinophilia was also noted.
What is your diagnosis?
This is an excellent example of Kimura's disease, a rather rare chronic inflammatory disorder of unclear pathogenesis. The clinical history in this case is quite typical; an Asian man with recurrent involvement of the deep subcutaneous tissues of the neck. Over the years there has been considerable confusion, particularly in the Western literature, of Kimura's disease with angiolymphoid hyperplasia with eosinophilia (histiocytoid / epithelioid hemangioma). The latter entity has different histologic features, particularly with regard to the nature of the endothelial cells, and does not show the marked ethnic and male prevalence of Kimura's disease.
Histologically, Kimura's disease is characterized by three components: 1. A cellular inflammatory infiltrate rich in eosinophils and associated with follicular lymphoid hyperplasia, 2. A proliferation of post-capillary venules, 3. A fibrocollagenous background.
Kimura's disease is a chronic disorder with an indolent clinical course, often waxing and waning over time. Patients typically have symptoms ranging from months to multiple years. The current patient had a 7-year history with three documented recurrences. As in the current case, peripheral eosinophilia is almost always present. Surgery is currently the mainstay of therapy, though steroids and radiation therapy have also been used. Recurrence after treatment is common.
The differential diagnosis of Kimura's disease is extensive and includes, at a minimum, Hodgkin's lymphoma, Langerhan's cell histiocytosis, Castleman's disease, florid lymphoid hyperplasia, angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma), parasitic infection, drug reactin, and Churg Strauss disease.
For an excellent review on this topic the reader is referred to the AFIP series of 21 cases published in AJSP 28:505-512,2004.