Maxillary sinus angiolymphoid hyperplasia with eosinophilia associated to orbital and cutaneous lesion.

INTRODUCTION: We describe the first case of ALHE in the orbit with extension to the maxillary sinus and the importance of a multidisciplinary approach to achieve removal of the lesion. CASE STUDY: A 72-year-old man presented with epiphora of the left eye and several episodes of recurrent acute dacryocystitis. Magnetic resonance imaging revealed a solid homogeneous mass located in the inferomedial region of the left orbit. In addition, it was associated with destruction of the adjacent ethmoidal wall and upper wall of the left maxillary sinus. Incisional biopsy of the orbital mass was compatible with ALHE. RESULTS: It was decided to perform surgery using an orbital floor approach, left medial wall via subconjunctival and caruncular approach together with an endoscopic nasal approach (ESS), achieving complete removal of the orbital mass and cleaning of the maxillary sinus. After one year of treatment, no tumor recurrence was evident through endoscopy and imaging tests and the patient is asymptomatic. CONCLUSIONS: ALHE is a very rare benign vascular tumor that presents subcutaneous nodules in the head and neck region. We do not know of any case of ALHE in the paranasal sinuses described in the literature, either in isolation or together with orbital or cutaneous ALHE. In conclusion, ALHE disease should be considered as a diagnosis when faced with an orbital mass with extension to the paranasal sinuses, and a complete excision through a combined endonasal and orbital approach prevents recurrence in most cases.

Coexisting Nodular Sclerosis Hodgkin Lymphoma and Kimura's Disease: A Case Report and Literature Review.

Kimura's disease (KD) is a rare lymphoproliferative fibroinflammatory disorder that commonly affects the subcutaneous tissue and lymph nodes of the head and neck. The condition is a reactive process involving T helper type 2 cytokines. Concurrent malignancies have not been described. Differential diagnosis with lymphoma can be challenging without tissue biopsy. Here, we present the first reported case of coexisting KD and eosinophilic nodular sclerosis Hodgkin lymphoma of the right cervical lymphatics in a 72-year-old Taiwanese man.

A case of membranous nephropathy with Kimura's disease.

BACKGROUND: The association between membranous nephropathy (MN) and Kimura's disease (KD) has been reported in recent years. The treatment, effect, and prognosis of KD are still unclear. CASE REPORT: A 47-year-old KD patient developed a left axillary mass for 3 years and received surgical resection because of the lager mass in August 2016. Then he developed nephrotic syndrome 3 months later. Laboratory index revealed increased eosinophil count, decreased albumin and heavy proteinuria. Lymph node biopsy suggested KD, and renal biopsy suggested MN. He relapsed after a treatment with methylprednisolone (52 mg/d) alone and then tacrolimus (1.5 mg/12h) was added. The patient had no symptoms of relapse in the next 2 years. CONCLUSION: The combination therapy of surgery, methylprednisolone, and immunosuppressive agents may be effective in KD with MN.

A Rare Kimura's Disease in the Oral Cavity with Severe Sleep Apnea: Case Report and Literature Review.

Kimura's disease (KD) is a rare chronic inflammatory disorder that commonly occurs in Asian males. It mainly presents as painless subcutaneous masses or lymphadenopathy in the head and neck region. The incidence of KD in the oral cavity is quite rare. We reported a rare case of a 53-year-old male who had KD in his soft palate, hard palate and bilateral tonsils associated with severe sleep apnea. This patient underwent radiotherapy and exhibited a good response to the treatment. Throughout the 12-month follow-up period, the patient's condition remained satisfactory. Of the other 14 reviewed cases of KD in the oral cavity, the lesions can occur in the buccal mucosa, hard and soft palate, and mouth floor with specific clinical features. We further summarized their manifestations and treatments in order to guide the future identification and management of KD with lesions in the oral cavity.

[Diagnostic challenges in Kimura's disease]. / Wyzwania w diagnostyce choroby Kimury.

Ultra-rare diseases occur with a frequency of 2 in 100 000 people or less. Kimura's disease (KD) affects less than 1 in 1 000 000 people. It is a benign, chronic inflammatory soft tissue disorder, accompanied by eosinophilia, raised immunoglobulin E (IgE) titer and the presence of painless subcutaneous masses, usually in the head and neck region. The disease was first described in 1948 and occurs at higher rates in Asia than in America or Europe. A CASE REPORT: A 35-year-old man without past medical history presented to his family doctor for bilateral cervical lymphadenopathy accompanied by eosinophilia. Despite subsequent in-depth diagnostics, including fine-needle aspiration biopsy (FNAB) of the lymph nodes, the definitive diagnosis was not initially established. After following 2 months, a selective lymphadenectomy was performed, putting Hodgkin's lymphoma under suspicion. The image of positron emission tomography coupled with computed tomography (PET-CT) corresponded to this diagnosis. Due to the lack of all the criteria necessary to make a diagnosis, another histopathological consultation was done. The image of the lymph nodes suggested reaction-inflammatory changes. Due to the presence of a triad of signs (reactive lymphadenopathy, several eosinophils in the paracortic zone, vascular proliferation), differential diagnosis was recommended, among others towards the Kimura's disease. A series of examinations allowed to exclude lymphadenopathy of parasitic, allergic and hyperplastic hematopoietic system aetiology. The patient started steroid therapy with a good effect at first. However, after the recurrence of the disease, the patient was qualified to intensify the immunosuppressive treatment. CONCLUSIONS: In the described case, the intensive diagnostic process and the thorough analysis of the test results relatively quickly led to the correct diagnosis. This enabled the implementation of appropriate treatment and prevented the initiation of empirical therapy for the originally diagnosed Hodgkin's lymphoma.

[Immune thrombocytopenia associated with Kimura's disease].

In this study, we report a rare case of immune thrombocytopenic purpura (ITP) associated with the worsening of Kimura's disease. A 47-year-old Japanese man with a pruritic rash and swollen inguinal lymph nodes was diagnosed with Kimura's disease on performing a right inguinal lymph node biopsy. Thrombocytopenia ensued after the diagnosis of Kimura's disease, and fluctuations in the platelet count were observed along with the pathology of Kimura's disease. The platelet count fluctuated repeatedly with the relapse of Kimura's disease and a diagnosis of a combination of Kimura's disease and ITP was made through lymph node regeneration and bone marrow examination. Treatment with prednisolone (1 mg/kg/day) was initiated for Kimura's disease and ITP, and lymphadenopathy and platelet count improved promptly. Since then, the dose of prednisolone has been gradually reduced, and the disease status of both Kimura's disease and ITP has been controlled.

Kimura's disease: a rare disease with cervical lymphadenopathy.

Kimura's disease is a very rare disorder and causes swellings and lymphadenopathy, especially in neck and axillary region. The aetiology is still unknown but allergic reactions or an alteration of immune system should be taken into consideration. Here, we report a 45-year-old Turkish male patient who presented to the Uludag University Medical Faculty Emergency Medicine Department with complaint of enlarged lymph nodes in the neck region and pruritus. His leukocyte count and eosinophil rate were found to be elevated in the emergency laboratory results. During his follow up the nodes were removed and histopathologically evaluated as Kimura's disease. In conclusion, one can say that the diagnosis of Kimura's disease can be very difficult and histopathological features play an important role. However, this diagnosis should not be underestimated in young male patients with lymph nodes in cervical area especially in the emergency department.

VEGF-A Links Angiolymphoid Hyperplasia With Eosinophilia (ALHE) to THSD7A Membranous Nephropathy: A Report of 2 Cases.

Autoantibodies against thrombospondin type 1 domain-containing 7A (THSD7A) cause membranous nephropathy (MN); however, the mechanisms involved in THSD7A expression and immunization are uncertain. We present 2 cases of THSD7A-associated MN accompanied by angiolymphoid hyperplasia with eosinophilia (ALHE), a benign tumor characterized by proliferation of plump endothelial cells. Prednisolone therapy, but not surgical resection of ALHE tumors, successfully suppressed eosinophilia and proteinuria in both cases. Because ALHE is characterized by the proliferation of plump endothelial cells, we focused on the roles of vascular endothelial growth factor A (VEGF-A) in MN pathogenesis. We found that plump endothelial cells in ALHE modestly expressed THSD7A in both cases. We also found that eosinophils in ALHE expressed VEGF-A, which upregulated THSD7A expression, especially under T-helper type 2-prone conditions in cultured endothelial cells. Furthermore, double-positive cells for THSD7A and CD83 surrounded the proliferated small vessels. Our results suggest that VEGF-A-induced THSD7A expression outside the kidney may be important for MN pathogenesis.

Kimura's disease with membranoproliferative glomerulonephritis: a case report with literature review.

BACKGROUND: Kimura's disease is a rare disease and its etiology is still unclear. Here we reported a case with lymphadenopathy complicated with secondary membranoproliferative glomerulonephritis. CASE PRESENTATION: A 46-year-old Chinese man presented with bilateral tumor-like nodules over his neck during the past 6 months and developed edema for 15 days. His blood pressure was 145/90 mmHg, multiple 1 × 1 cm masses were found over bilateral post-auricular and submandibular areas, along with trace edema of the lower extremities. Laboratory data showed an increased peripheral eosinophil count at 3.66 × 109/L (50% of total leukocytes), with a 24-hour urine total protein of 8 g and a serum albumin of 19 g/L, and serum IgE of 2930 IU/ml (<100 IU/ml). The patient underwent renal biopsy, which revealed membranoproliferative glomerulonephritis with eosinophilic infiltration of the interstitium. Lymph node biopsy showed eosinophilic lymphoid follicular granuloma. Bone marrow biopsy showed no abnormalities. A diagnosis of Kimura's disease was then established. We started him on prednisone 60 mg/day (1 mg/kg), and tapered the dose to 55 mg/day 2 months later, followed by a gradual reduction of 2.5 mg every 2 weeks. Valsartan was given for blood pressure control. His neck nodules shrank after 2 weeks of treatment and complete renal remission was achieved 3 months later. No relapse occurred after follow-up for 31 months. CONCLUSION: Kimura's disease can present with bilateral neck nodules and nephrotic syndrome (membranoproliferative glomerulonephritis), and prednisone can be a suitable choice of treatment.

Kimura's Disease.

Kimura's disease (KD) is a benign, rare and chronic inflammatory disorder of unknown etiology and it affects subcutaneous tissues, lymph nodes, and salivary glands. The clinical manifestation of cases reported is mainly small neoplasms or enlarged lymph nodes, while cases for large masses are rarely reported. This case reveals a female patient of KD with large masses in the head and neck region. The scope of the tumor is huge, which seriously affects the facial shape and psychology of the patient, thereby affecting the daily life of the patient. Although the patient had surgically removed the same site tumor 8 years ago, it was not possible to determine whether it was a recurrent case because the postoperative pathological diagnosis of first treatment was not confirmed. Under the general anesthesia, the right head and neck masses were removed. During the operation, the facial nerve branches were wrapped by the masses. After consulting the family members, it was agreed that the facial nerve should not be preserved for maximum extent removal of the tumor. Although the patient has facial paralysis symptoms after surgery, the facial shape and patient's psychology are greatly improved, and the patient is satisfied with the treatment effect. Surgical treatment is one of the effective ways to treat huge tumors in the head and neck of KD. This study has certain guiding significance for clinicians to treat huge tumors of head and neck in KD.

Nephrotic syndrome associated with Kimura's disease: a case report and literature review.

BACKGROUND: Kimura's disease (KD) is a rare chronic inflammatory disorder with a high incidence of renal involvement. In this report, we present a case study of KD-associated nephrotic syndrome combined with minimal change disease (MCD) and acute renal tubular injury. Meanwhile, the clinical and histopathological characteristics of 26 patients with KD presenting with renal involvement were retrospectively evaluated. CASE PRESENTATION: Here, we report a case study of a 59-year-old male patient with KD confirmed by a lymph node biopsy. He developed widespread edema and decreased urine output. A palpable swollen mobile and non-tender lymph node behind the left ear was observed upon admission. A renal biopsy revealed minimal-change lesions and acute renal tubular injury. The patient received hemodialysis because of the oliguria and renal insufficiency, and an initial dose of 40 mg/d methylprednisolone and then continued treatment with 40 mg/d prednisolone. He exhibited a good clinical response to the steroid after 6 weeks of treatment. Of the other 26 patients included in the review, 13 patients presented with mesangial proliferative glomerulonephritis, 4 with membranous nephropathy, 3 with MCD, 3 with focal segmental glomerulosclerosis, 2 with IgA nephropathy and 1 with acute tubular injury. With the exception of 2 patients who progressed to end-stage renal disease and received hemodialysis, the majority of patients responded well to treatment with corticosteroids alone. CONCLUSIONS: MCD combined with acute renal tubular injury is rare in patients with KD presenting with renal involvement. Corticosteroids may be a beneficial treatment for renal injury in patients with KD.

Three unusual histopathological presentations of angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a poorly understood benign vasculoproliferative disorder. Histopathologically, the lesions are composed of a dermal or subcutaneous proliferation of blood vessels of varying sizes with plump and bland endothelial cells, which often show vacuolization and protrusion of the cytoplasm into the lumen. The vascular proliferation is admixed with a diffuse inflammatory infiltrate composed of lymphocytes, eosinophils and mast cells. Lymphoid follicles with germinal centers can sometimes be seen. In addition, fibrosis is a common accompanying feature. Some clinical and histopathological variants of ALHE have already been described in the literature. In this report, we present 3 rare associations of ALHE that have not been previously described. Case 1 was a 73-year-old woman with a lesion on her right medial thigh. Examination showed ALHE admixed with a chronic lymphocytic leukemic (CLL) infiltrate. Case 2 was a 55-year-old woman with a lesion on her right anterior ankle, which was a syringocystadenoma papilliferum co-existing with an ALHE. Case 3 was a 54-year-old man with a lesion on the left medial thigh, which showed overlapping features of IgG4-related disease associated with areas of ALHE. Given these multiple and diverse associations, it seems likely that ALHE may be a reactive rather than neoplastic process.

Successful therapy with mycophenolic acid in a membranous glomerulonephritis due to Kimura disease
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Kimura disease (KD) is a rare inflammatory soft tissue disorder of unknown origin most frequent in Asians, the prevalence of which is growing in Western countries. Painless papules and/or nodules with a predilection for the head and the neck region, lymphadenopathies, parotid gland involvement, eosinophilia, and raised IgE levels are parts of its presentation. Renal involvement with various forms of glomerulonephritis, including membranous nephropathy (MN), can occur and is generally associated with a proteinuria that encompasses nephrotic syndrome. Corticosteroids are the mainstay of treatment of KD-associated glomerulonephritis, but steroids withdrawal is often followed by relapsing nephrotic syndrome. Various immunosuppressive agents have been tested to prolong the remission of KD-associated nephrotic syndrome while tapering steroids, but they are only partly effective or associated with significant complications. To the best of our knowledge, we describe here the first case of KD-related membranous glomerulonephritis with a favorable evolution and a sustained remission of 4 years under prolonged therapy with mycophenolic acid (MPA). MPA and its active metabolite, mycophenolate mofetil (MMF), treatments as supportive therapies to corticosteroids and ACE inhibitors should be further investigated in KD-related membranous nephropathies.
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Eosinophilic hyperplastic lymphogranuloma: Clinical diagnosis and treatment experience of 41 cases.

PURPOSE: The purpose of this study was to investigate the clinical features of eosinophilic hyperplastic lymphogranuloma (EHLG) in the head and neck. MATERIALS AND METHODS: Collecting the patients who diagnose with EHLG by pathological examination. The EHLG patients with the masses involved regions, such as involved inguinal region, chest wall, abdominal wall, anterior superior iliac spine or clavicle, instead of head and neck were excluding. All of the participants will sign the informed consent form. The history data includes: clinical history, blood routine test, pathological examination, and recurrence will be collected. RESULTS: A total of 41 patients of EHLG were included. These patients predominantly presented as an enlarging and painless single or multiple masses with a history of repeated swelling. There were the complaint of itchy skin and pigmentation. The routine blood test showed that the percentage value of eosinophil increased in almost patients including 26 cases had raised absolute eosinophil count. The serum level of lgE was increased in 29 cases remarkably. With the methods of treatments, 36 patients received surgical excision, 3 patients accepted hormonotherapy, and another 2 patients for radiotherapy. The recurrence of EHLG was in 9 patients. CONCLUSIONS: EHLG is a rare disease. The clinical manifestation (itchy skin and pigmentation) and increased eosinophil play critical values to the diagnosis of EHLG. Confirmed diagnosis always depends on pathological examination. Surgery is a preferred treatment, while low dose of radiotherapy is necessary for preventing relapse after operation and hormonotherapy.

Clinicopathological features and prognosis of Kimura's disease with renal involvement in Chinese patients.

AIMS: Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment. MATERIALS AND METHODS: Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively. RESULTS: The patients consisted of 27 males and 2 females, with an average age of 35.5 ± 15.1 (13 - 61) years. 27 exhibited proteinuria ranging from 0.730 to 14.1 g/24 h (5.98 ± 3.40 g/24 h). Hypertension, renal insufficiency (serum creatinine (Scr) > 1.24 mg/dL), and microhematuria occurred in 4 (13.8%), 11 (37.9%), and 13 (44.8%) cases, respectively. Light microscopy (LM) identified mesangium proliferation, minimal change, focal and segmental glomerulosclerosis (FSGS), membranous glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), and acute tubular necrosis in 14, 8, 3, 2, 1, and 1 cases, respectively. All were treated with Tripterygium wilfordii (TW), prednisone, leflunomide (LEF), tacrolimus (FK506), myophenolate mofetil (MMF), or renin-angiotensin system blockers (RASI). 26 patients were followed up for 1.60 - 108.7 months (39.6 ± 28.7). After treatments, urinary red blood cells (RBC) decreased in all. The amount of 24-hour urinary protein (24-hUPE) decreased in 24 patients. 22 reached complete remission (CR), 4 partial remissions (PR). The patients who did not relapse were younger than those who relapsed. CONCLUSIONS: KD with renal involvement occurs predominantly among 35 - 50 year old Chinese patients with male predilection. The most common features are proteinuria, hypertension, micro hematuria with minimal change, and mesangial proliferative glomerulonephritis. Most were responsive to treatment, but could relapse. Gender, age, and hypertension are associated with KD recurrence. The prognosis is good mostly.

Kimura's disease: A case presentation of postauricular swelling.

Kimura's disease (KD) is a rare chronic inflammatory disease of subcutaneous tissues and occurs predominantly in head and neck region. It is seen primarily in young Asian males. Typical clinical presentations are painless subcutaneous masses, regional lymph node enlargement, blood and tissue hypereosinophilia, and increased serum IgE levels. Here, we present a case of a 27-year-old female who presented with unilateral single nodular swelling in the right postauricular region. The diagnosis of KD was done based on characteristic histopathologic finding in conjunction with peripheral eosinophilia and increase in serum IgE levels.

Kimura's disease associated necrotizing eosinophilic vasculitis presenting with recurrent peripheral arterial occlusive disease: a case report and review of the literature.

A 33-year-old Chinese man with 9-year history of Kimura's disease (KD) was admitted with a 1-month history of recurrent claudication. He did not have any clinical discomfort and had not taken any preventive medication in the past. He accepted percutaneous transluminal angioplasty and the pathologic diagnosis was reportedly consistent with necrotizing eosinophilic vasculitis. This is the rare reported case of KD associated necrotizing eosinophilic vasculitis presenting with recurrent peripheral arterial occlusive disease and the difficulties encountered in establishing an accurate diagnosis with unusual presentations. This case also highlights the possibility of recurrent complications without aggressive medical treatment in such unusual eosinophilic disorders.

Diagnosis and treatment of a patient with Kimura's disease associated with nephrotic syndrome and lymphadenopathy of the epitrochlear nodes.

BACKGROUND: Kimura's disease (KD) is a slowly progressing rare, benign inflammatory disorder of the soft tissues. It typically presents as subcutaneous tumor-like nodules, located most frequently in the head and neck region. KD is often accompanied by increased peripheral eosinophilia and elevated levels of serum immunoglobulin (Ig) E. There is renal involvement in approximately 12-16% of KD cases. We report the case of a 23-year-old Chinese man who was found to have KD associated with nephrotic syndrome. CASE PRESENTATION: A 23-year-old Chinese man presented with edema in both legs and a mass in ulnar side of his right upper arm on August 8(th) 2013. Before admission to our hospital, an ultrasound examination revealed swollen lymph nodes in the medial aspect of his right upper arm, proximal to the elbow. The patient was admitted on August 19(th) 2013 as a result of edema, severe proteinuria, and low serum albumin levels. He had a white blood cell count of 7.7 × 10(9) cells/L, 48.5% eosinophils, 4+ albuminuria, 24-hour urinary protein excretion 9.3 g, serum protein 50.3 g/L; serum albumin 16 g/L and IgE 1,510 IU/ml. A biopsy of the epitrochlear nodes revealed eosinophilic hyperplastic lymphogranulomatous tissue. A renal biopsy indicated focal segmental glomerulosclerosis (FSGS) (cellular variant) with no infiltration of eosinophil in renal interstitium. The results of immune-staining on the renal biopsy were negative for IgG, IgA, IgM, C3 and C1q. The electron microscopic analysis showed podocyte effacement. His final diagnosis was Kimura's disease associated with nephrotic syndrome. He received methylprednisolone therapy as well as symptomatic treatment, and was discharged with key indicators in normal range on September 17(th) 2013. During the year following, he had methylprednisolone at a maintenance dose of 8 mg/day, and no relapses occurred up to now. CONCLUSION: Methylprednisolone therapy is effective in KD associated with nephrotic syndrome, and long-term administration of methylprednisolone at maintenance dose may be a way to prevent relapses of KD.