Kimura's disease involving the ipsilateral face and extraocular muscles

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 x 1.5 cm(2) 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.

Keywords: Exophthalmos; Extraocular muscle; Eyelid swelling; Kimura's disease.

Fig. 1

Photograph of patient's face prior to surgery showing exophthalmos of the left eye and swelling of the left upper eyelid and buccal area.

Fig. 2

T2-weighted MRI scan with enhancement showed exophthalmos of the left eye and diffuse thickening of the left extraocular muscles (A, B) and a relatively well-defined enhancing homogenous mass at the left masseter muscle, left temporalis muscle, and left medial pterygoid muscle (C).

Fig. 3

A&B, Histopathological examination of an incisional biopsy of the left lateral rectus muscle showed lymphoid cell aggregation and abnormal vascular proliferation with marked eosinophilic infiltration in hypertrophied collagenous tissue. (A: Hematoxylin & Eosin ×100, B: Hematoxylin & Eosin ×200).

Fig. 4

After two months of oral steroid therapy, the exophthalmos of the left eye and facial swelling dramatically decreased, as documented by this photograph of the patient's face following therapy.

Fig. 5

A&B, Histopathological examination of the excised facial mass showed follicular hyperplasia and abnormal vascular proliferation with massive mature eosionphilic infiltration in hypertrophied collagenous tissue. (A: Hematoxylin & Eosin ×40, B: Hematoxylin & Eosin ×200).