Treatment of Kimura's disease with cyclosporine
Division of Dermatology, Department of Laboratory Medicine, Changi General Hospital, Singapore. Journal of Dermatological Treatment
(Impact Factor: 1.67).
02/2005; 16(4):242-4. DOI: 10.1080/09546630510044003
Kimura's disease is a rare, chronic inflammatory disease of unknown cause. It is characterized by painless subcutaneous swellings and lymphadenopathy, commonly affecting the head and neck region. This is associated with peripheral blood eosinophilia and raised serum IgE. It has distinct histological features of lymphoid follicles, eosinophilic infiltrate, fibrosis and vascular proliferation. The disease usually has a benign, indolent course. Traditionally, therapeutic options have included surgery, radiotherapy and steroids but response has been less than satisfactory. Recently, cyclosporine has been reported to be effective in the treatment of Kimura's disease. In this article, we present a middle-aged Chinese female with Kimura's disease for 20 years and her favourable response to cyclosporine.
Available from: Sang Joon Lee
- "Recurrence of KD frequently follows incomplete excision. Shenoy et al. reported that cyclosporine produces an excellent response in recurrent KD.17 Cyclosporine has also been reported to be effective in the treatment of KD.18 In our case, the patient was treated with an extraocular muscle incision biopsy and facial mass excision combined with postoperative oral corticosteroid therapy. "
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ABSTRACT: Kimura's disease (KD) is a rare, chronic inflammatory disorder, which is characterized by tumor-like masses mainly located in the head and neck region. Extraocular muscle involvement in KD is uncommon. We report a case of KD that involved both the extraocular muscles and buccal area. A 13-year-old male presented to our clinic with a two-year history of exophthalmos of the left eye and facial swelling. Facial CT and MRI showed a 1.5 × 1.5 cm2 soft tissue mass located at the left masticator and buccal area, exophthalmos of the left eye, and diffuse thickening of the left extraocular muscles. We performed a lateral rectus muscle incisional biopsy of the left eye. Oral methylprednisolone therapy was initiated and tapered following the incisional biopsy.
Histopathologic findings of the lateral rectus muscle incisional biopsy showed abnormal vascular proliferation with marked eosinophilic infiltration in hypertrophied collagenous tissue. Post-operative histopathologic findings of the facial mass confirmed the diagnosis of KD.
Although KD with extraocular muscle involvement is uncommon, an ophthalmologist can diagnose KD by the clinical presentation of exophthalmos, eyelid swelling, and an orbital massas well as by histological examination of a biopsy of the orbital mass.
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ABSTRACT: Our objective was to retrospectively analyze the clinical and histological features of 8 Chinese patients with Kimura's disease and renal involvement.
Eight male patients with Kimura's disease admitted to Jinling Hospital from 1998 to 2006 were included. The diagnoses were confirmed by lymphoid biopsy. Peripheral blood eosinophil, CD4+ and CD8+ T lymphoid cell count and total serum IgE level were examined. Renal biopsy, IL-4 expression cells and IgE-positive cell counts in renal tissue were performed. Follow-up data were recorded in detail.
Six patients were observed with the onset symptoms of subcutaneous mass or enlarged lymph nodes, and the other 2 with edema. Constitutional symptoms included bronchial asthma, enteritis, eczema, neuritis and nephrotic syndrome. Renal biopsy revealed mesangial proliferation with or without IgA deposition under immunofluorescence (n=6) and membranous nephropathy (n=2). Tubulointerstitial infiltration of eosinophils was found in 6 patients. IL-4-positive cells in renal interstitium were detected in 5 cases. IgE-positive cells were negative in all cases. Fusion of the epithelial foot process was observed under electron microscopy in patients with mesangial proliferation. All patients were sensitive to treatment with systemic prednisone, but 4 patients had a renal or extrarenal relapse after the dose tapered. Three patients who presented with solitary masses and underwent lymphoidectomy or mass excision were free from renal and extrarenal relapse. All patients had normal renal function at last follow-up.
Mesangial proliferation, eosinophilic infiltration and podocyte confusion are prominent histological features of this cohort of patients. Patients are sensitive to prednisone therapy but apt to relapse. Lymphoidectomy can be helpful to prevent relapse.
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