Kimura disease is a chronic inflammatory disorder prevalent in Asians. It involves subcutaneous tissues and lymph nodes predominantly in the head and neck region and is characterized by angiolymphoid proliferation and eosinophilia.

Kimura disease; eosinophilic lymphogranuloma.

Endemic in Asia and sporadic in other geographic regions.

Kimura disease is suggestive of an infectious origin, but no pathogen has been demonstrated.

The disease, described in 1948 by Kimura and coworkers (1) and earlier reported in a Chinese medical journal (2), is endemic in Asia, occurring primarily in China, Japan, and Indonesia (3). In the West, Kimura disease is sporadic and was for a time considered to be the late stage of a condition called angiolymphoid hyperplasia with eosinophilia (ALHE), described by Wells and Whimster in 1969 (4). Subsequent studies made the distinction between the two entities, as delineated in the differential diagnosis (3,5,6,7,8).

In the United States, the largest, comprehensive study originating at the Armed Forces Institute of Pathology (AFIP) was published in 2004 (9).

Essentially, Kimura disease is located deep in the subcutaneous tissues and in almost all cases involves the regional lymph nodes; in contrast, ALHE is largely restricted to the dermis (3,8,10). It occurs predominantly in young adults; the age range is 27 to 40 years and the male-to-female ratio is 3:1. The onset is insidious, and the manifestations are enlarging nodular masses in the head and neck areas, most frequently infra- or retroauricular, which may simulate a neoplasm. The lesions are single in 60% of cases and multiple, occasionally symmetric, in the rest (6). Occasionally, an isolated enlarged lymph node is the only presentation (3). Occasionally, the salivary glands, particularly the parotid, oral cavity, axilla, groin, and limbs, are involved (11). Cases have also been reported of eosinophilic infiltrates in skeletal muscles, prostate, and kidney, the latter resulting in a nephrotic syndrome (3,11,12). Peripheral blood eosinophilia—10% to 50%—and elevated serum immunoglobulin E (IgE) levels are constant features of Kimura disease (3,6). The skin and lymph node lesions do not ulcerate and, although recurrences are common, the disease has a benign course (2,11). Best clinical results are obtained with radiation therapy since both surgical excision and steroid treatments have high rates of recurrence (13,14).