Kimura's disease successively affecting multiple body parts: a case-based literature review

Abstract

Background: Kimura's disease is a rare, benign, chronic inflammatory disease that presents as painless, solid masses mainly affecting the deep subcutaneous areas of the head and neck, especially the salivary glands, parotid glands and nearby lymph nodes. It is characterized by elevated peripheral blood eosinophil and Immunoglobulin E (IgE) levels.

Case presentation: A 31-year-old Asian male presented with an orbital space-occupying lesion lasting for 1.5 years. Ten years prior, surgical excision of bilateral fossa cubitalis and groin masses was performed, and the pathological examination showed "lymphoproliferative disease". One year later, masses reappeared near the surgical sites; they grew slowly and shrank after glucocorticoid treatment. At this point, admission examinations showed in the peripheral blood an eosinophil proportion of 13.4%, a total IgE level of 26,900.00 IU/mL, prurigo present on the whole body, and multiple palpable masses near the bilateral fossa cubitalis and groin. The left eyeball was exophthalmic. The left elbow mass was excised, and the pathological examination confirmed Kimura's disease. Oral glucocorticoid therapy is taken and tapering regularly. The eosinophil count returned to normal, the IgE level gradually decreased, the orbital space-occupying lesion and elbow and groin masses shrank significantly, and the whole-body skin prurigo disappeared. Currently, the patient has been in a stable condition for eighteen months.

Conclusion: Our case provides a novel insight that Kimura's disease should be involved in the differential diagnosis of inflammatory lesion mass of orbit and also supports systemic regular glucocorticoid as a valuable therapy of such condition, but close follow-up and long-term observation are crucial.

Keywords: Eosinophils; Fossa Cubitalis; Groin; Immunoglobulin E; Kimura’s disease; Orbit.

Fig. 1

Orbital CT. A shows the lesion mass is medium density. The deep part is adhered to the optic nerve (sagittal scan); B shows part lesion invades the infraorbital fissure (coronal scan); C shows the lesion is adjacent to the medial rectus muscle and is inhomogeneous contrast enhancement (horizontal enhanced scan); D reveals the subcutaneous thickening (horizontal enhanced scan)

Fig. 2

Orbital MRI. T1 weighted image shows the lesion is low signal (A, orbital horizontal scan); T2 weighted image shows medium signal (B, orbital sagittal scan); The lesion mass is significantly enhanced (C, orbital sagittal enhanced scan); Part lesion is adhesion to the inferior rectus muscle and optic nerve (B and C)

Fig. 3

The lower abdomen CT. A and B reveal multiple nodular masses in the groin on both sides; C and D reveal inhomogeneous contrast enhancement in the arterial phase

Fig. 4

The left orbital colour Doppler ultrasound. The orbital subnasal lesion mass is in the size of 2.16*1.37 cm, and has a heterogeneous hypoechoic-isoechoic appearance; the blood flow is relatively rich

Fig. 5

The Colour Doppler ultrasound of bilateral elbow. The bilateral elbow masses are oval in shape and have a heterogeneous hypoechoic-isoechoic appearance; the blood flows are relatively rich. (A is the right elbow; B is the left elbow)

Fig. 6

Ocular Appearance. (A) shows the left eyeball protopsis at the time of intravenous drip of methylprednisolone (40 mg) for one day; (B) shows the left eyeball protopsis is significantly alleviative at the time of intravenous drip of methylprednisolone (40 mg) for five days

Fig. 7

Surgical removal of the left elbow mass. The mass was further for pathological examination

Fig. 8

The pathological characteristics of elbow mass. A The gross view of pathological sections. In the cortical region, hyperplasia of numerous lymphoid follicles with active germinal centers (× 20); B Magnification of the marked region with the rectangle in (A). Strong eosinophilic infiltration in the interfollicular area, accompanied by postcapillary venule proliferation with intermingled fibrotic changes. (× 100); C Magnification of the marked region with the rectangle in (B). Eosinophilic microabscesses (× 400)