RESUMO
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.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Imageamento por Ressonância Magnética , Seio Maxilar , Humanos , Idoso , Masculino , Hiperplasia Angiolinfoide com Eosinofilia/cirurgia , Hiperplasia Angiolinfoide com Eosinofilia/patologia , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Seio Maxilar/patologia , Seio Maxilar/diagnóstico por imagem , Seio Maxilar/cirurgia , Endoscopia/métodos , Doenças Orbitárias/cirurgia , Doenças Orbitárias/patologia , Doenças Orbitárias/diagnóstico por imagem , Doenças Orbitárias/etiologiaRESUMO
BACKGROUND: Kimura disease (KD) is a rare chronic inflammatory disorder involving the Th2 pathway. Although medical treatment with steroids or other immunosuppressants is available, they may cause developmental issues in the pediatric population. Surgical intervention has also been suggested; however, it is associated with high recurrence rates. CASE PRESENTATION: A 14-year-old boy presented with left retroauricular lymph node enlargement at the age of 5 years. At the age of 7 years, he was diagnosed with nephrotic syndrome which subsided after steroid treatment for approximately 6 years. The retroauricular lymph node was surgically excised, and KD was confirmed. However, recurrent enlargement of the left retroauricular and neck lymph nodes occurred after 2 years. Persistently high IgE levels and fluctuating eosinophil counts were observed following steroid treatment. Dupilumab was prescribed because of the difficulty in tapering the steroid dosage. A loading dose of 600 mg was administered, followed by a maintenance dose of 300 mg every 2 weeks. The IgE level decreased after 3 months, and a low eosinophil count was maintained after steroid discontinuation. Follow-up computed tomography revealed a decrease in the size of the lymph nodes with no side effects such as conjunctivitis. CONCLUSION: Traditional treatments have raised developmental concerns in the pediatric population and are associated with high recurrence rates. Dupilumab targets the Th2 pathway and provides effective results, with few adverse effects. Dupilumab may be a therapeutic option for KD and other diseases involving the Th2 pathway.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Doença de Kimura , Masculino , Humanos , Criança , Pré-Escolar , Adolescente , Doença de Kimura/complicações , Doença de Kimura/tratamento farmacológico , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/tratamento farmacológico , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Esteroides/uso terapêutico , Imunoglobulina ERESUMO
Epithelioid hemangioma is a benign vascular neoplasm with a characteristic histological and immunohistochemical pattern, characterized by a lymphocytic inflammatory infiltrate with admixed eosinophils and FOSB expression. The correct diagnosis is of particular relevance, since malignant vascular tumors with differentiated epithelioid cells can also be considered in the differential diagnosis. We present a patient with multiple epithelioid hemangiomas of the scalp accompanied by severe pain and itching. The long history of multiple therapeutic attempts illustrates the limited success of currently available treatment options.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Hemangioma , Neoplasias Vasculares , Humanos , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Couro Cabeludo/patologia , Hemangioma/diagnóstico , Diagnóstico Diferencial , Neoplasias Vasculares/complicaçõesRESUMO
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.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Doença de Hodgkin , Doença de Kimura , Masculino , Humanos , Idoso , Doença de Kimura/diagnóstico , Doença de Kimura/patologia , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/patologia , Doença de Hodgkin/complicações , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/patologia , Esclerose/patologia , Linfonodos/patologia , Diagnóstico Diferencial , Doenças Raras/diagnósticoRESUMO
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.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Doença de Kimura , Masculino , Humanos , Pessoa de Meia-Idade , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/patologia , Doença de Kimura/complicações , Doença de Kimura/patologia , Palato Duro/patologia , Mucosa Bucal/patologia , Doenças Raras/patologiaRESUMO
Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare nodular mass that has not commonly been reported in the literature in association with coccidioidomycosis (CM). Coccidioidomycosis has other known skin manifestations including erythema nodosum and interstitial granulomatous dermatitis. Pulmonary CM is the most common form of the disease and the most common cause of CM-associated rash. This is an important clinical consideration for patients with ALHE who reside in CM-endemic areas, which notably include the southwestern region of the United States, Mexico, and South America. We report the case of an ALHE lesion that resolved following treatment for CM.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Coccidioidomicose , Hemangioma , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/patologia , Coccidioidomicose/complicações , Coccidioidomicose/diagnóstico , Hemangioma/complicações , Humanos , MéxicoRESUMO
Kimura's disease is a rare, benign entity that causes subcutaneous angioblastic lymphoid hyperplasia with eosinophilia. It usually presents with subcutaneous lymphoid swellings with regional lymphadenopathy and salivary gland masses. Kimura's disease is frequently associated with renal involvement, which includes proteinuria and nephrotic syndrome as the most common presentations. This report presents a case of a man in his early 20s with a swelling in the medial aspect of the distal left arm with two previous episodes of nephrotic syndrome. Multiple enlarged axillary lymph nodes and epitrochlear nodes were noted. Ultrasonography revealed a mixed echogenic mass with enlarged lymph nodes. MRI showed a heterogeneous hyperintense lesion. Image-guided fine-needle aspiration showed multiple lymphocytes and eosinophils suggestive of Kimura's disease. The swelling was surgically excised under general anaesthesia. Histopathology of the excised mass confirmed the diagnosis of Kimura's disease.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Doença de Kimura , Linfadenopatia , Síndrome Nefrótica , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/cirurgia , Braço/patologia , Dor no Peito/patologia , Humanos , Doença de Kimura/complicações , Doença de Kimura/diagnóstico , Doença de Kimura/cirurgia , Linfonodos/patologia , Linfadenopatia/diagnóstico por imagem , Linfadenopatia/patologia , Masculino , Síndrome Nefrótica/complicaçõesRESUMO
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.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Doença de Hodgkin , Doença de Kimura , Linfadenopatia , Adulto , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/terapia , Doença de Hodgkin/complicações , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/terapia , Humanos , Doença de Kimura/diagnóstico , Linfadenopatia/complicações , Linfadenopatia/etiologia , Masculino , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/efeitos adversosRESUMO
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.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Glomerulonefrite Membranosa , Doença de Kimura , Síndrome Nefrótica , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/tratamento farmacológico , Glomerulonefrite Membranosa/complicações , Glomerulonefrite Membranosa/diagnóstico , Glomerulonefrite Membranosa/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/etiologia , TacrolimoRESUMO
BACKGROUND: Kimura disease is a rare inflammatory condition classically manifested as painless subcutaneous nodules in the head and neck region and associated with regional cervical lymphadenopathy and salivary gland involvement. OBJECTIVE: The purpose of this report is to illustrate the diagnostic difficulty due to its rarity and non-specific clinical presentation. CASE PRESENTATION: We present a case of Kimura disease with bilateral eyelid swelling, parotid involvement, and cervical lymphadenopathy in a young boy. Computed Tomography (CT) showed ill- defined enhancing masses in both upper eyelids with lacrimal gland infiltration, multiple nodular lesions in both parotid glands, and bilateral enlargement of cervical nodes. After several multidisciplinary discussions and trials of medications, Kimura's disease was finally confirmed by histology examination. CONCLUSION: In conclusion, a rare disease such as Kimura must be considered earlier if the patient is not responding to the treatment.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Neoplasias de Cabeça e Pescoço , Doença de Kimura , Linfadenopatia , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico por imagem , Criança , Neoplasias de Cabeça e Pescoço/complicações , Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Humanos , Linfadenopatia/diagnóstico por imagem , Linfadenopatia/etiologia , Masculino , Pescoço/diagnóstico por imagem , Pescoço/patologiaRESUMO
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.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Doença de Kimura , Linfadenopatia , Púrpura Trombocitopênica Idiopática , Trombocitopenia , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/tratamento farmacológicoAssuntos
Antagonistas Adrenérgicos beta/administração & dosagem , Hiperplasia Angiolinfoide com Eosinofilia/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Paroniquia/terapia , Timolol/administração & dosagem , Administração Tópica , Idoso , Hiperplasia Angiolinfoide com Eosinofilia/induzido quimicamente , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Antibacterianos/administração & dosagem , Terapia Combinada/métodos , Crioterapia/métodos , Receptores ErbB/antagonistas & inibidores , Feminino , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Paroniquia/induzido quimicamente , Paroniquia/complicações , Paroniquia/diagnóstico , Inibidores de Proteínas Quinases/efeitos adversos , Estudos Retrospectivos , Índice de Gravidade de Doença , Nitrato de Prata/administração & dosagem , Resultado do TratamentoRESUMO
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.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia , Doença de Kimura , Linfadenopatia , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Humanos , Linfonodos , Linfadenopatia/etiologia , Masculino , Pessoa de Meia-Idade , Doenças RarasRESUMO
RATIONALE: Ankylosing spondylitis (AS) and Kimura's disease (KD) which is quite rare are both chronic inflammatory diseases. Recently we encountered a patient who suffered from KD and AS, and some of his family members also suffer from AS. We, therefore, investigated this unique case and conducted the family-based whole exome sequencing to explore the possible genetic alterations. PATIENT CONCERNS: Here, we reported a case of a 44-year-old Chinese man with multiple painless masses all over his body and a back pain for 32 years. His uncle and sister were diagnosed with AS. DIAGNOSIS: The diagnosis of KD was based on the patient's clinical features and the biopsy of the neck masses. The diagnosis of AS was based on the patient's clinical features, HLA-B27(+) and the radiologic changes of sacroiliac joints. The genetic test showed that ARPC1B gene which was associated with recurrent infections, auto-inflammatory changes and elevated IgE levels was mutated in this patient. INTERVENTIONS: Neck masses were removed by surgery. Systemic glucocorticoid, nonsteroidal anti-inflammatory agents, combined with cyclosporine were orally administered, and Etanercept was injected subcutaneously. OUTCOMES: The masses disappeared rapidly after surgery combined with systemic glucocorticoid, but relapsed shortly after the therapy was discontinued. Low dose glucocorticoid, cyclosporine and Etanercept could keep both KD and AS remained long-term remission. LESSONS: Our experience suggests that low dose glucocorticoid, cyclosporine and Etanercept could be beneficial for the patient with KD and AS. The mutation of ARPC1B gene in this case, which is associated with immunologic disturbance, may increase the susceptibility of KD.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia/complicações , Espondilite Anquilosante/complicações , Adulto , Hiperplasia Angiolinfoide com Eosinofilia/genética , Humanos , Masculino , Espondilite Anquilosante/genéticaAssuntos
Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Linfadenopatia/diagnóstico , Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/patologia , Pré-Escolar , Eosinofilia/sangue , Humanos , Imunoglobulina E/sangue , Japão , Linfadenopatia/complicações , Linfadenopatia/patologia , MasculinoAssuntos
Hiperplasia Angiolinfoide com Eosinofilia/complicações , Oclusão da Artéria Retiniana/etiologia , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico , Criança , Exoftalmia/diagnóstico por imagem , Humanos , Masculino , Órbita/diagnóstico por imagem , Oclusão da Artéria Retiniana/diagnóstico , Tomografia Computadorizada por Raios X , Acuidade VisualRESUMO
BACKGROUND: Kimura disease (KD) is a chronic benign inflammatory disorder that is usually manifested as a deep, subcutaneous mass with or without regional lymphadenopathy in the head and neck region. Various types of glomerulonephritis are associated with KD, including minimal change disease (MCD), membranous glomerulopathy (MN), and immunoglobulin (Ig)A-nephropathy. Kidney interstitial lesion associated with KD is rarely reported. The aim of this study was to expand the spectrum of kidney lesions associated with KD. METHODS: From 2007 to 2016, 12 cases of KD with kidney lesions were retrospectively reviewed. Pathological examinations included hematoxylin and eosin staining, periodic acid-schiff staining, periodic acid-methenamine silver staining, and Masson staining, immunofluorescence, and electron microscope analyses. RESULTS: Anatomic sites of subcutaneous involvement included head and neck area, arm, and groin. Most cases had elevated IgE level and peripheral eosinophilia. Nephrotic syndrome was the most common kidney manifestation. Pathological results showed 3 types of glomerulonephritis, including 9 cases of MCD, 2 cases of IgA nephropathy, and 1 case of MN. Of note, 4 MCD cases showed concurrent glomerular and interstitial lesions associated with KD, including 2 distinct patterns: (1) a diffusely eosinophilic and lymphatic infiltration similar to KD lesion developed elsewhere and (2) exclusively eosinophilic infiltration in the interstitium. Eight out of 10 patients were responsive to steroid treatment and had complete remission of proteinuria and recovery of kidney function. CONCLUSION: Our report suggested that, in addition to glomerulonephritis, interstitial lesions may also be associated with KD. Pathologist should pay special attention to differentiated diagnosis when such pathological changes are identified in patients with subcutaneous mass or cervical lymphadenopathy with concurrent kidney manifestation.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/patologia , Nefropatias/complicações , Nefropatias/patologia , Adolescente , Adulto , Hiperplasia Angiolinfoide com Eosinofilia/tratamento farmacológico , Estudos de Coortes , Diagnóstico Diferencial , Feminino , Glomerulonefrite/complicações , Glomerulonefrite/patologia , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/patologia , Glomerulonefrite Membranosa/complicações , Glomerulonefrite Membranosa/patologia , Glucocorticoides/uso terapêutico , Humanos , Nefropatias/tratamento farmacológico , Glomérulos Renais/patologia , Masculino , Pessoa de Meia-Idade , Nefrite Intersticial/complicações , Nefrite Intersticial/patologia , Nefrose Lipoide/complicações , Nefrose Lipoide/patologia , Síndrome Nefrótica/complicações , Síndrome Nefrótica/patologia , Estudos Retrospectivos , Adulto JovemRESUMO
Lymphadenopathy along with various systemic manifestations is commonly encountered in pediatric patients, tuberculosis being the commonest etiology. Occasional patients may present a diagnostic conundrum. Here, the authors report an unusual manifestation of Kimura disease (KD) presenting as nephrotic syndrome associated with mesenteric lymphadenitis in an 11-year-old male child. KD is a chronic inflammatory disorder of unknown etiology. It typically affects young adult males in the age range of 27-40 years and usually presents as painless itchy nodular masses in the head and neck region. The involvement of mesenteric lymph nodes along with a very young age of presentation makes it a rare case, posing a diagnostic challenge for the unsuspecting physician.
Assuntos
Hiperplasia Angiolinfoide com Eosinofilia/complicações , Hiperplasia Angiolinfoide com Eosinofilia/diagnóstico por imagem , Linfadenite Mesentérica/etiologia , Síndrome Nefrótica/diagnóstico por imagem , Abdome/diagnóstico por imagem , Anti-Inflamatórios/uso terapêutico , Criança , Diagnóstico Diferencial , Humanos , Linfonodos/patologia , Imageamento por Ressonância Magnética , Masculino , Síndrome Nefrótica/complicações , Prednisona/uso terapêutico , UltrassonografiaRESUMO
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.