Kimura disease accompanied with Nephrotic syndrome in a 45-year-old male
© Gong et al.; licensee BioMed Central. 2015
Received: 31 October 2014
Accepted: 16 April 2015
Published: 28 April 2015
Kimura disease (KD) is an uncommon chronic inflammatory disorder of unknown etiology, occurs mainly in Asian young males, presenting as subcutaneous growing masses, with a predilection for head and neck, with or without satellite lymphadenopathy. Herein, we report a case of an atypical manifestation of KD accompanied with NS in a middle-aged man, though the patient was clinically misdiagnosed previously. The diagnosis of KD can be difficult and misleading, so we must explore the main points of KD so as to prevent misdiagnosis.
Letter to the editor
KD is an uncommon chronic inflammatory disorder of unknown cause, involving subcutaneous tissue, predominantly in the head and neck region. It can be associated with lymphadenopathy (both local and distant), obvious peripheral blood eosinophilia, and an elevated IgE level . Most cases reported to date involve young Asian males, with most patients being aged between 20 and 40 years . The incidence of KD with coexisting renal disease ranges from 10% to 60% [3,4]. Herein, we report an atypical manifestation of KD accompanied with nephrotic syndrome (NS) occurring in a 45-year-old man.
A 45-year-old Chinese man came to our department with a soft mass in medial aspect of his right upper arm. According to the patient, the mass appeared in 2003 for the first time and that was without any obvious cause. He became aware of the mass after about 5 years when he noticed that the enlargement was gradually progressive. Then he visited general surgery department in October 2008 and underwent surgical resection of mass after 3 months in January 2009.
The histopathology report of the mass at that time revealed a proliferation of lymphoid follicles and diffuse infiltration of eosinophil, and WBC: 8200/dL (eosinophil: 38%). So it was diagnosed provisionally as a suspected case of parasitic infection by a physician, and then the patient wasn’t followed up.
The final diagnosis was KD accompanied with NS. But the patient refused the renal biopsy. After integrity surgical excision of the mass, he was treated with oral prednisone 30 mg per day for 4 months and responded well with normalization of eosinophil counts and IgE values, disappearance of proteinuria after 3 months. In addition, the enlarged LN shrunk to normal after 4 months. Currently, the patient is only on a low dose of oral prednisone (7.5 mg/d). There has been no recurrence of the disease for 1 year.
Association of KD with renal disease in the form of proteinuria and NS is well recognized. Proteinuria may occur in 12–16% of patients and 59–78% of them have a NS [3,4]. The renal pathologies reported in China have included minimal change disease, mesangioproliferative and membranous nephropathy, while a wider spectrum of histological lesions have been reported in other countries . In our case, the patient refused the renal biopsy, so we didn’t know renal pathology.
A comparison of the clinical and histological features of KD with ALHE
Angiolymphoid hyperplasia with eosinophilia
Female predominance (70%)
Male predominance (85%)
Young to middle age
More common in Asians
Occurs in all races
Head and neck
Head and neck
Localized subcutaneous mass
Dermal papules or nodules
Single or multiple
Average 3 cm
Average 1 cm
Lymph node involvement
Almost invariably present
Serum immunoglobulin E (IgE) level
Some degree of vascular proliferation
Florid vascular proliferation
May be present
Sparse to abundant
Cuboidal to dome shaped:"Histiocytoid"
Treatment for KD includes surgical resection and regional or systemic steroid therapy . Cytotoxic therapy and irradiation have also been utilized. Surgical excision of the lesion(s) is the first line therapy but relapses are frequent . Systemic corticotherapy with prednisone is prescribed when renal involvement is present, but with a risk of relapse on withdrawal of medicine. In our case, our patient who is a middle-aged man presented with atypical KD with renal involvement that responded well to prednisone without relapse. The treatment resulted in rapid remission of NS along with the normalization of eosinophil count and IgE levels. The dose of prednisone had been tapered to 7.5 mg/d for 7 months and disease had not relapsed. He is still being followed-up.
Written informed consent was obtained from the patient for the publication of this report and any accompanying images.
All the authors of this article and the patient in the text.
Source(s) of support
This work was supported in parts by grants from the Science and Technology Commission of Shanghai Municipality Grant (134119b0700).
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