Open Access

Kimura disease accompanied with Nephrotic syndrome in a 45-year-old male

Contributed equally
Diagnostic Pathology201510:43

https://doi.org/10.1186/s13000-015-0277-1

Received: 31 October 2014

Accepted: 16 April 2015

Published: 28 April 2015

Abstract

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.

Keywords

Kimura disease Nephrotic syndrome Angiolymphoid hyperplasia with eosinophilia Prednisone

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 [1]. Most cases reported to date involve young Asian males, with most patients being aged between 20 and 40 years [2]. 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.

In 2013, the lesion reappeared and gradually became larger than before, so he visited our clinic. Physical examination revealed a firm, nontender, mobile, subcutaneous mass of 4 cm × 5 cm (Figure 1). The overlying skin was normal except for a 5 cm long scar (Figure 1). The patient’s blood pressure was 135/90 mmHg. The laboratory values were as follows: creatinine (2.4 mg/dL), normal electrolytes, low serum albumin (28 g/L), hypertriglyceridemia (3.2 mmol/L), proteinuria (3.5 g/d), WBC (8360/dL), eosinophilia (42% ), haemoglobin (13.6 g/dL), IgE (17100 IU/ml). Antinuclear antibody and other disease specific autoantibodies were negative. The bilateral lymph nodes (LN) of neck and axillae were enlarged. Investigations for a haematological malignancy including immunophenotyping of circulating lymphocytes, search for clonal T cell population and FIP1L1-PDGFRA fusion gene mutation, and examination of a bone marrow biopsy all gave negative results.
Figure 1

Clinical appearance of the case: a nontender firm subcutaneous swelling of 4 cm × 5 cm in size in the right upper arm region (arrow).

Histological examination of a biopsy sample showed a proliferation of lymphoid follicles with distinct germinal centres (Figure 2a), capillary proliferation (Figure 2b), and diffuse infiltration of eosinophil and eosinophilic microabscess (Figure 2c and d).
Figure 2

Histologic examination of a biopsy sample from the subcutaneous mass of the upper arm (H and E): (a) proliferation of lymphoid follicles with distinct germinal centres (arrow; × 40); (b) vascular proliferation (arrow; × 100); (c, d) formation of eosinophilic microabscess and intense eosinophilic infiltration (arrow; c × 200; d × 400).

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 [5]. In our case, the patient refused the renal biopsy, so we didn’t know renal pathology.

KD is often confused with malignancies, T cell lymphoma, Hodgkin’s disease, parasitic infection and histiocytosis X. Ultrasound, CT, and magnetic resonance imaging (MRI) might be diagnostic and can help staging the extent and progression of the disease as well as the lymph node involvement. The peripheral blood eosinophilia can also mimic a parasitic infection or an allergic reaction. However, the lesion that bears the closest resemblance to KD is Angiolymphoid Hyperplasia with Eosinophilia (ALHE). The clinical and histological features of KD and ALHE are compared in Table 1 [6,7].
Table 1

A comparison of the clinical and histological features of KD with ALHE

 

Kimura disease

Angiolymphoid hyperplasia with eosinophilia

clinical features

  

Sex

Female predominance (70%)

Male predominance (85%)

Age

Young adulthood

Young to middle age

Race

More common in Asians

Occurs in all races

Location

Head and neck

Head and neck

Presentation

Localized subcutaneous mass

Dermal papules or nodules

Number

Single or multiple

Usually multiple

Size

Average 3 cm

Average 1 cm

Lymph node involvement

Common

Rare

peripheral eosinophilia

Almost invariably present

Rare (20%)

Serum immunoglobulin E (IgE) level

Elevated

Normal

Renal involvement

Occasional (21%)

Rare

Recurrence rate

30%

15-40%

Histopathological features

  

Depth

Subcutaneous, muscle

Cutaneous, subcutaneous

Vascular proliferation

Some degree of vascular proliferation

Florid vascular proliferation

Lymphoid follicles

Always found

May be present

Eosinophils

Abundant

Sparse to abundant

Eosinophils abscesses

Present

Not seen

Endothelium

Flattened

Cuboidal to dome shaped:"Histiocytoid"

Fibrosis

Present

Absent

Treatment for KD includes surgical resection and regional or systemic steroid therapy [8]. Cytotoxic therapy and irradiation have also been utilized. Surgical excision of the lesion(s) is the first line therapy but relapses are frequent [5]. 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.

Consent

Written informed consent was obtained from the patient for the publication of this report and any accompanying images.

Notes

Declarations

Acknowledgement

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).

Authors’ Affiliations

(1)
Department of Dermatology, Shanghai Tenth People’s Hospital, Tongji University School of Medicine
(2)
Department of Dermatology, Tongji University School of Medicine

References

  1. Messina MTD, Armstrong WB, Pena F, Allison G, Kim JKV. Kimura’s disease: two case reports and a literature review. Annals of Otology, Rhinology and Laryngology. 1998;107(12):1066–71.View ArticleGoogle Scholar
  2. Sorbello M, Laudini A, Morello G, Sidoti MT, Maugeri JG, Giaquinta A, et al. Anaesthesiological implications of Kimura’s disease: a case report. J Med Case Rep. 2009;3:7316.View ArticlePubMed CentralPubMedGoogle Scholar
  3. Park SW, Kim HJ, Sung KJ, Lee JH, Park IS. Kimura disease: CT and MR imaging findings. AJNR Am J Neuroradiol. 2012;33(4):784–8.View ArticlePubMedGoogle Scholar
  4. Fouda MA, Gheith O, Refaie A, El-Saeed M, Bakr A, Wafa E, et al. Kimura Disease: A case report and review of the literature with a new management protocol. Int J Nephrol. 2010;2011:673908.Google Scholar
  5. Liu C, Hu W, Chen H, Tang Z, Zeng C, Liu Z, et al. Clinical and pathological study of Kimura’s disease with renal involvement. J Nephrol. 2008;21:517–25.PubMedGoogle Scholar
  6. Dik VK, van der Wiel BA, Vasmel WL. Kimura's disease of the parotid glands and multiple cervical lymph nodes. Neth J Med. 2010;68:175–7.PubMedGoogle Scholar
  7. Sah P, Kamath A, Aramanadka C, Radhakrishnan R. Kimura's disease - An unusual presentation involving subcutaneous tissue, parotid gland and lymph node. J Oral Maxillofac Pathol. 2013;17(3):455–9.View ArticlePubMed CentralPubMedGoogle Scholar
  8. Tseng CF, Lin HC, Huang SC, Su CY. Kimura's disease presenting as bilateral parotid masses. Eur Arch Otorhinolaryngol. 2005;262(1):8–10.View ArticlePubMedGoogle Scholar

Copyright

© Gong et al.; licensee BioMed Central. 2015

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Advertisement