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Editor,—Kimura’s disease is an uncommon, chronic inflammatory disorder of unknown aetiology which occurs predominantly in orientals and presents with tumour-like swellings mainly in the head and neck region. The condition primarily involves the subcutaneous tissues, parotid glands, and/or lymph nodes. Orbital cases are infrequent and most reported cases are in adults, with only one case in a child of Afro-Caribbean descent.1Our case report presents orbital Kimura’s disease in an 8 year old white child.
CASE REPORT
An 8 year old white girl presented to us with a 4 week history of a painless swelling of her right upper lid. She initially had an upper respiratory tract infection lasting a month which was treated by her general practitioner with Augmentin. She subsequently developed what appeared to be a right sided ptosis. Of note, she had a strong history of allergic eye disease with intermittent conjunctivitis, and also suffers from asthma for which she takes Pulmicort and Bricanyl inhaler regularly. Examination revealed a right partial ptosis, mild right proptosis, and a palpable, painless mass in the superior aspect of her right orbit. There were no pupillary abnormalities, her extraocular movements were full, and funduscopic examination was unremarkable. There was no associated regional lymphadenopathy and the rest of the ocular and general physical examination showed no other abnormalities.
Investigations done included a full blood count, which was within normal limits. WBC 8.57 ×109/l with a differential of neutrophils 62%, lymphocytes 26%, monocytes 6%, eosinophils 5%, and basophils 1%. A magnetic resonance imaging (MRI) scan with gadolinium contrast was done which showed an extensive superior orbital mass which was not clearly delineated, but involved the upper eyelid (see Fig 1). An orbital biopsy was subsequently performed. Histological sections revealed fibrosis with a marked vascular proliferation and a prominent perivascular inflammation consisting of eosinophils and lymphocytes (see Fig 2). There were also scattered lymphocytes and eosinophils throughout the fibrotic tissue with reactive lymphoid follicles. There was no evidence of necrosis, granulomas, or malignant cells. Immunohistochemical studies revealed no cells of myogenous origin present. Studies for desmin, myoglobin, and vimentin were negative. All of the cells considered lymphoid on the haematoxylin and eosin stain also stained for the leucocyte marker common leucocyte antigen (CLA). A diagnosis of Kimura’s disease (angiolymphoid hyperplasia with eosinophilia) was made.
A magnetic resonance imaging (MRI) scan with contrast. There is an extensive superior orbital mass on the right side.
Section showing vascular proliferation with eosinophilic and lymphocytic perivascular inflammation and fibrosis. Haematoxylin and eosin stain. Original magnification ×200.
At biopsy, the tumour was debulked and the patient was thereafter placed on a short 6 week course of oral steroids. The ptosis resolved and at 1 year follow up, there has been no clinical evidence of recurrence.
COMMENT
Kimura’s disease (or angiolymphoid hyperplasia with eosinophilia) is a chronic inflammatory disorder of unknown aetiology which presents with tumour-like swellings mainly in the head and neck region. Most cases have been described in China and Japan, with relatively fewer cases reported in non-orientals.2 The disease typically affects males in the 20–40 year age group, and presents as single or multiple smooth swellings in the subcutaneous tissues, major salivary gland and/or lymph nodes in the head and neck area.
Histopathologically, the features described in previous cases3 4 corresponded with ours; there are eosinophilic infiltrates (mainly in a perivascular pattern), vascular proliferation, fibrosis, and formation of lymphoid follicles. It is still uncertain as to whether it represents a benign lymphoid neoplastic process, a variant of haemangioma or an allergic reaction. There have been no reported cases of malignant change or fatalities. There is usually an insidious onset with a long benign course, but recurrences are common after surgical excision. Systemic associations include asthma and nephrotic syndrome.3 5 Regional lymphadenopathy ranges from 50–75% of cases and there is a high occurrence of peripheral blood eosinophilia.
Cases of Kimura’s disease have been reported in the orbits5 6; however, there was only one previously reported orbital case in a child (of Afro-Caribbean descent).1 To our knowledge, this is the first orbital case reported in a white child. Non-orbital cases of Kimura’s disease have been treated with surgical excision, irradiation and steroid therapy. After active therapy, they tend to recur. Cases have been conservatively managed for up to 12 years successfully1 although the cosmetic deformity can be quite a handicap. Periorbital cases have so far been treated successfully with surgical excision and biopsy with debulking.5 6Oral steroids or intralesional injection with steroids have not been as successful. There was only one reported case of severe haemorrhage on surgical resection.1 Complete surgical excision appears to be the best treatment.