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.
Hinweise
Yu Gong and Jun-Ying Gu contributed equally to this work.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
Jun-Ying Gu, Yu Gong and Yu-Ling Shi participated in conception of the idea, Yu Gong and Jun-Ying Gu have been involved in drafting the manuscript. Yu Gong and Sony Labh has been involved in revising it critically for important intellectual content. All authors performed the pathological and immunohistochemical interpretation of the tumor tissue. All authors have read and approved the final manuscript.
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.
Anzeige
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.
×
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).
×
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.
Anzeige
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.
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).
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made.
The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder.
The authors declare that they have no competing interests.
Authors' contributions
Jun-Ying Gu, Yu Gong and Yu-Ling Shi participated in conception of the idea, Yu Gong and Jun-Ying Gu have been involved in drafting the manuscript. Yu Gong and Sony Labh has been involved in revising it critically for important intellectual content. All authors performed the pathological and immunohistochemical interpretation of the tumor tissue. All authors have read and approved the final manuscript.
Um auch an kleinen Gewebeproben zuverlässige und reproduzierbare Ergebnisse zu gewährleisten ist eine strenge Qualitätskontrolle in jedem Schritt des Arbeitsablaufs erforderlich. Eine nicht ordnungsgemäße Prüfung oder Behandlung des …
Die vergleichende experimentelle Pathologie („comparative experimental pathology“) ist ein Fachbereich an der Schnittstelle von Human- und Veterinärmedizin. Sie widmet sich der vergleichenden Erforschung von Gemeinsamkeiten und Unterschieden von …
Gastrointestinale Stromatumoren (GIST) stellen seit über 20 Jahren ein Paradigma für die zielgerichtete Therapie mit Tyrosinkinaseinhibitoren dar. Eine elementare Voraussetzung für eine mögliche neoadjuvante oder adjuvante Behandlung bei …
Aufgrund des erheblichen technologischen Fortschritts in der molekularen und genetischen Diagnostik sowie zunehmender Erkenntnisse über die molekulare Pathogenese von Krankheiten hat in den letzten zwei Jahrzehnten ein grundlegender …