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Increasing awareness of KD in Western clinicians by finding pathognomonic features
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KD should be considered in the differential diagnoses of head and neck masses.
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Definitive diagnosis can be obtained by histological examination of excised lesion.
1. Introduction
A 32-year-old Pakistani man without significant comorbidity presented to the Emergency Department with generalized edema. Physical examination highlighted lower limbs and scrotum swelling with tense-elastic, indolent, bilateral retroauricular masses (Fig. 1a ). Blood tests showed severe peripheral eosinophilia (WBC 9500/μL, E 2650/μL, confirmed by peripheral blood smear), high levels of IgE (7710 U/mL), severe hypoalbuminemia (1.2 g/dL), dyslipidemia (LDL cholesterol 266 mg/dL) with mild renal impairment (eGFR 40 mL/min/1.73 m2). The 24 h urine collection pointed out severe proteinuria (19–23 g/24 h), confirming the hypothesis of nephrotic syndrome. Serum transaminases, gamma-glutamyl transferase, alkaline phosphatase, coagulation markers and pseudocholinesterase were normal. The screening for the commonest viral, bacterial, parasitic and fungal infections of our country, rheumatologic antibodies, and serum/urine immunofixation all resulted negative. The total body CT scan showed no important abnormalities. It was therefore performed excisional biopsy of the right retroauricular mass, which showed hyperplastic lymphoid tissue, inflammatory infiltrate rich in eosinophils and overgrowth of endothelial cells and histocytes (Fig. 1b–c).
Fig. 1(a) Tense-elastic right retroauricular mass. (b) Excisional biopsy of the retroauricular right mass. (c) Hystologic findings. (d) 1-month follow up: disappearance of retroauricular swelling after corticosteroid therapy.
In consideration of geographical origin of the patient, physical examination, laboratory and histologic findings was diagnosed Kimura Disease (KD). KD is a rare chronic inflammatory disorder of unknown cause, described for the first time in China in 1937 as “eosinophilic hyperplastic lymphogranuloma”; it most commonly presents as painless subcutaneous masses in the head and neck region, frequently associated with nephrotic syndrome, peripheral eosinophilia and high levels of serum IgE. It has been hypothesized that an infection or toxin may trigger an autoimmune phenomenon or lead to an IgE-mediated hypersensitivity reaction. The exact prevalence of KD is not known (<1/1.000.000), with most cases reported in Southeast Asia and only two documented cases in Italy.
After excisional resection of the retroauricular mass we initiated treatment with high doses of methylprednisone (1.5 mg/kg/die), with the patient presenting a clinical improvement consisted in reduction of peripheral edema and retroauricular masses and progressive improvement of the laboratory findings (proteinuria 3.2 g/24 h, serum albumin 1.8 g/dL, eosinophils 20/μL, IgE 5240 U/mL). At discharge we recommended high doses of methylprednisone, to taper off. At 1-month follow-up, while the patient was still tapering steroids, we documented the normalization of the clinical (Fig. 1d) and a significant improvement of serum albumin levels (3 g/dL), IgE (2790 U/mL) and proteinuria (0.69 g/24 h); a 3-month follow up (after suspension of therapy) showed complete normalization of laboratory findings.
Funding source
None.
Conflict of interest
The authors state that they have no conflicts of interest.
Authorship
Both authors had access to the data and contributed to writing the manuscript.
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