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]

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 (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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Differential Diagnoses

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Media Gallery

High-magnification micrograph of Kimura disease, hematoxylin and eosin stain. Thick-walled blood vessels with (plump) hobnail endothelial cells and abundant eosinophils. Courtesy of Nephron (own work) via Wikimedia Commons.
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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Author

Alan Snyder MD Candidate, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Daniel S Loo, MD Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center

Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology, Association of Professors of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Takeji Nishikawa, MD Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc

Disclosure: Nothing to disclose.

Alaina J James, MD, PhD Assistant Professor, Department of Dermatology, The Hospital of the University of Pennsylvania

Alaina J James, MD, PhD is a member of the following medical societies: American Academy of Dermatology, National Medical Association, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Evan W Piette, MD Resident Physician, Department of Dermatology, Hospital of the University of Pennsylvania

Evan W Piette, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Sena J Lee, MD, PhD Resident Physician, Department of Dermatology, Hospital of University of Pennsylvania

Disclosure: Nothing to disclose.

Patricia Mercado, MD Associate Professor of Dermatology, University of Alabama at Birmingham School of Medicine

Disclosure: Nothing to disclose.

Laura M Tamburin, MD Affiliated Dermatology, PC

Disclosure: Nothing to disclose.

Karolyn A Wanat, MD Resident Physician, Department of Dermatology, University of Pennsylvania School of Medicine

Karolyn A Wanat, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and American Medical Women's Association

Disclosure: Nothing to disclose.