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.
"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. "
[Show abstract][Hide abstract] 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.
Korean Journal of Ophthalmology 09/2009; 23(3):219-23. DOI:10.3341/kjo.2009.23.3.219
[Show abstract][Hide abstract] ABSTRACT: Kimura disease (KD) is a rare, chronic inflammatory disease of unknown cause and is characterized by painless s.c. swellings and lymphadenopathy commonly affecting the head and neck region. Much therapeutics has been used to treat KD, but is not satisfactory because of frequent relapse. Imatinib has been reported previously to be useful for treatment of hypereosinophilic syndrome and may work by selectively blocking protein-tyrosine kinases, such as platelet-derived growth factor receptor, and c-Kit. We carried out immunohistochemical examination of platelet-derived growth factor receptor-alpha and c-Kit in tissues from patients with KD. The results were positive and suggested that Imatinib might be an effective drug for the treatment of the disease. We have also briefly reviewed the epidemiology, aetiology, clinical manifestations, laboratory and pathological examinations, differential diagnoses, treatment and prognosis of KD in this manuscript.
Internal Medicine Journal 09/2008; 38(8):668-72. DOI:10.1111/j.1445-5994.2008.01711.x · 1.64 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Kimura's disease is a chronic inflammatory disorder of unknown etiology. A 14 year old boy suffering from steroid dependant nephrotic syndrome, was presented with relapsing painless subcutaneous masses on the left buccal area. Blood analysis showed increased IgE and eosinophilia. During 4 years follow up, he was been treated by low dose steroid and short term cyclosporine. Consequently, frequent relapses of subcutaneous masses and nephrotic syndrome has been relieved. Cyclosporine treatment combined with steroid may be useful for preventing frequent relapse of Kimura's disease.
Journal of the Korean Society of Pediatric Nephrology 01/2009; 13(2). DOI:10.3339/jkspn.2009.13.2.271
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