Kimura Disease Clinical Presentation

Updated: Mar 15, 2022
  • Author: Alan Snyder; Chief Editor: William D James, MD  more...
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Presentation

History

Kimura disease typically presents as a painless mass or masses in the head and neck region, with occasional pruritus of the overlying skin. Renal disease, nephrotic syndrome in particular, is present in up to 20% of patients with Kimura disease. [28] An estimated 12-16% of patients with Kimura disease exhibit proteinuria upon examination, of which 59-78% have nephrotic syndrome. [29] Less commonly, several reports in the literature have linked Kimura disease with a hypercoagulable state in patients without associated nephrotic syndrome. [30]

In a study of 238 patients with an average age of 36 years, Kakehi et al found no significant differences related to age in complications, anatomic distribution, maximum size, eosinophil count, immunoglobulin E level, recurrence, or outcomes. [27]  

Tomizuka et al raise the question of whether there is an association between Kimura disease and juvenile temporal arteritis. Both diseases are eosinophilic inflammatory conditions, and a literature review of 48 juvenile temporal arteritis cases found Kimura disease as a copathology in 6 cases. [31]

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Physical Examination

Patients with Kimura disease typically present with nontender subcutaneous nodules and masses in the head and neck, especially in the parotid and submandibular regions. These lesions are often associated with lymphadenopathy. Less frequently, the orbit (including the eyelids, conjunctiva, and lacrimal glands [32] ), paranasal sinuses, epiglottis, tympanic membrane, parotid gland, parapharyngeal space, palate, axilla, groin, and breast [25, 33, 34, 35] may be involved. [36, 37] Although Kimura disease mainly affects the head and neck, involvement of the extremities and inguinal lymph nodes has been reported. [38] In addition, a presentation of Kimura disease as a pulmonary hilar mass has been described. [39] Visible ischemia of the extremities may be present as a result of the hypercoagulable sequelae of Kimura disease, and related chronic diseases such as Raynaud phenomenon and acute limb ischemia (ALI) have been reported when left untreated. [7, 40, 41]

See the image below.

Fifteen-year-old boy with nontender, firm swelling Fifteen-year-old boy with nontender, firm swelling (5 cm x 3 cm) in the left cervical area of 8 months’ duration. Courtesy of Our Dermatology Online (http://www.odermatol.com/).
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