Sections

Kimura Disease

Treatment

Medical Care

Observation is acceptable if the Kimura disease lesions are neither symptomatic nor disfiguring.

Oral corticosteroids are commonly used; however, the disease frequently recurs after cessation of therapy. Intralesional corticosteroids may be effective for localized disease.^[17]

Oral corticosteroids in combination with cetirizine may prove to be an effective alternative treatment to surgery to reduce related nodular masses. In one patient, continued daily cetirizine prevented recurrence 4 months after tapering steroids.^[33]

Leflunomide in combination with oral prednisone may be an option for treating patients with or without renal involvement who are unresponsive to corticosteroids alone. The subject of 1 study remained disease free at the 12-month follow up after treatment with leflunomide and methylprednisone.^[48]Leflunomide may have an antiproliferative effect on eosinophils.

Cyclosporine has been reported to induce remission in patients with Kimura disease.^{[49, 50, 51]}A dose of 5 mg/kg/d was effective, but the lesions may recur upon cessation of therapy.^[52]

Intravenous immunoglobulin (IVIG) was used in 1 patient as a steroid-sparing agent, and he remained disease-free more than 6 years after follow-up.^[53]

Oral pentoxifylline has been reported to be effective in 1 patient with Kimura disease; however, the lesions relapsed after discontinuation of therapy.^[54]

Dupilumab has been reported to be effective in 1 patient with Kimura disease after surgical excision. The patient was started on dupilumab 600 mg followed by 300 mg every 2 weeks for 8 months. No recurrence was seen in 1 year of followup.^[55]

All trans-retinoic acid in combination with prednisone has resulted in remission of Kimura disease in 1 patient, and he remained disease free 12 months after discontinuation of all therapy.^[56]

Imatinib may be an effective treatment for Kimura disease, based on advances in research for therapy in hypereosinophilic syndrome, but further investigation is necessary.^[19]

Photodynamic therapy has been used successfully in 1 patient who experienced recurrence of disease after initial surgical management.^[13]

Radiotherapy has occasionally been used to treat recurrent or persistent Kimura disease lesions. A report by Hareyama et al^[57]described the use of radiotherapy at dosages of 26-30 Gy; local control was achieved in 74% of lesions. Another study demonstrated that radiotherapy (20-45 Gy) was more effective than local excision and steroid treatment, with local response rates of 64.3% versus 22.2%, respectively. No adverse effects were observed during a mean follow-up period of 65 months.^[58]New technology such as 3-dimensional printing is being explored for preventing collateral damage during radiotherapy bolus delivery to radiosensitive areas such as the head and neck.^[59]However, considering the benign nature of Kimura disease, radiation should be reserved for recurrent or disfiguring lesions.

The largest retrospective meta-analysis of Kimura disease treatment to date (n=639) concluded that surgical resection with low-dose postoperative radiotherapy resulted in the lowest recurrence rate of Kimura disease. Furthermore, it suggested that corticosteroid therapy should be a second-line option for treatment, owing to the potential adverse effects of long-term corticosteroid use and the high rates of recurrence when used alone.^[60]The same conclusions were made in another study comparing recurrence rates in 46 patients who underwent steroid therapy, surgical excision, radiotherapy, or surgical excision with radiotherapy.^[17]

Medication

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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.

Kimura Disease Treatment & Management

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