Kimura disease.Kimura disease is a rare, chronic inflammatory disorder that involves subcutaneous tissues, predominantly those in the head and neck region. Its etiology is unknown, but it is frequently associated with regional lymphadenopathy lymphadenopathy /lym·phad·e·nop·a·thy/ (-op´ah-the) disease of the lymph nodes. angioimmunoblastic lymphadenopathy , angioimmunoblastic lymphadenopathy with dysproteinemia and/or salivary gland involvement. Kimura disease has a predilection for males of Asian descent. Clinically, it can simulate a neoplasm neoplasm or tumor, tissue composed of cells that grow in an abnormal way. Normal tissue is growth-limited, i.e., cell reproduction is equal to cell death. , and most patients have peripheral blood eosinophilia eosinophilia /eo·sin·o·phil·ia/ (e?o-sin?o-fil´e-ah) abnormally increased eosinophils in the blood. e·o·sin·o·phil·i·a n. An increase in the number of eosinophils in the blood. and elevated serum immunoglobulin E (IgE) levels. These solitary masses invariably affect lymph nodes and subcutaneous soft tissues. Their characteristic histologic features include preserved nodal architecture, florid follicular fol·lic·u·lar adj. 1. Relating to, having, or resembling a follicle or follicles. 2. Affecting or growing out of a follicle or follicles. hyperplasia with reactive germinal centers, protein and IgE deposits in germinal centers, germinal center necrosis, eosinophilic eosinophilic /eo·sin·o·phil·ic/ (-fil´ik) 1. readily stainable with eosin. 2. pertaining to eosinophils. 3. pertaining to or characterized by eosinophilia. infiltrates, proliferation of post-capillary venules venules (vēnˑ·yōōlz), n.pl small blood vessels that merge with the veins and return blood from other tissues to the heart. , sclerosis, polykaryocytes, eosinophilic folliculolysis, and prominent eosinophilic microabscesses (figure). Kimura disease is sometimes confused with angiolymphoid hyperplasia with eosinophilia, Hodgkin lymphoma, angioimmunoblastic T-cell lymphoma Angioimmunoblastic T-cell lymphoma (AILT) is a mature T-cell lymphoma with systemic characterized by a polymorphous lymph node infiltrate showing a marked increase in follicular dendritic cells (FDCs) and high endothelial venules (HEVs) and systemic involvement. , florid follicular hyperplasia, Castleman disease, and lymphadenopathy secondary to drug reactions and parasitic infections. Appropriate cultures, histochemical stains, and immunophenotypic studies will help separate these unique and distinct lesions. Kimura disease is a chronic disorder--usually a localized process without systemic symptoms--that follows an indolent clinical course. Itchiness, urticaria, and chronic eczema, along with renal disease, are occasionally associated. Surgery is the mainstay of therapy, although regional or systemic corticosteroid therapy, cytotoxic therapy, and radiation have been used. Recurrence after surgery or following discontinuation of steroid treatment is common. Suggested reading Hui PK, Chart JK, Ng CS, et al. Lymphadenopathy of Kimura's disease. Am J Surg Pathol 1989;13:177-86. Ioachim HL, Ratch H. Kimura lymphadenopathy. In: Ioachim HL, Ratch H, eds. Ioachim's Lymph Node Pathology. Philadelphia: Lippincott-Raven, 2002:209-11. From the Department of Pathology, Woodland Hills Medical Center, Southern California Kaiser Permanente Group, Woodland Hills, Calif. |
|
||||||||||||||||||
Printer friendly
Cite/link
Email
Feedback
Reader Opinion