Two cases of dupilumab-responsive Kimura disease.

Kimura disease (KD) is a rare, chronic angiolymphoproliferative inflammatory disease appearing to be mostly restricted to the skin and soft tissue. Cutaneous involvement of KD includes head and/or neck nodules showing suggestive histological features, frequently associated with an atopic dermatitis-like or prurigo-like presentation. KD is challenging to treat, with high rate of recurrence using current therapeutic strategies. Evidence for involvement of a T-helper type 2 (Th2) immune response in KD pathogenesis has been found in previous studies. Consequently, this study aimed to determine the efficacy and safety of dupilumab, a human monoclonal antibody that inhibits signalling of key Th2 cytokines, interleukin (IL)-4 and IL-13, within a single-centre cohort of patients with cutaneous KD. Two adults with a diagnosis of refractory (failure of at least one treatment line) cutaneous-restricted KD based on clinical, biological, histological, molecular and imaging findings received dupilumab for KD, and showed dramatic response with a good safety profile.

Diagnostic challenges and updated therapeutic strategies of Kimura's disease: A case report successfully treated by dupilumab and review.

RATIONALE: Kimura's disease (KD) is a rare and chronic eosinophilic related-disease, characterized by subcutaneous tissue masses, regional enlarged lymph nodes, hypereosinophilia and elevated serum IgE. KD usually affects young adults in the Asian population. In Western countries, the clinical and biological presentation of KD is often unknown, delaying the diagnosis. Therapeutic management is not standardized and despite recent advances, remission from KD can be difficult to achieve, especially in relapse situations. PATIENT CONCERNS: We report the case of an non-Asian man with KD, initially misdiagnosed as lymphoma. We focus on his long-lasting clinical course with 20 years of recurrence despite several therapeutic lines. DIAGNOSES AND INTERVENTIONS: We have emphasized the key points of the KD diagnostic challenge. We chose to focus on hemopathies as diagnostic traps to illustrate several overlapping features that blur frontiers with KD. With regard to treatments, lessons can be learned from the use of the therapeutic backbone, which relies on excision surgery, radiotherapy and corticosteroids. OUTCOMES: Advancements in KD pathogenesis have highlighted the pivotal role of Th2 lymphocytes driving eosinophil activation. Directly inspired by eosinophilic and allergic field practices, targeted therapies, such as dupilumab, provide hope for potential curative options. LESSONS: Finally, we propose a therapeutic plan to treat newly diagnosed KD and discuss options for relapsing entities.

Bilateral swelling of the salivary glands and sicca symptoms: an unusual differential diagnosis-Kimura's disease, a rare allergic condition with a high IgE serum level-a case report and review of the literature.

A 68-year-old woman presented with bilateral swelling of the salivary glands, sicca symptoms of eyes and mouth, itching, fatigue and weight gain of about 5 kg in the last 2-3 years. As part of a careful diagnostic work up including lab tests for antinuclear antibodies (ANA), antibodies to extractable nuclear antigens (ENA), anti-neutrophilic cytoplasmatic antiobodies (ANCA), immunoglobulin (Ig)G4, a whole body computed tomography (CT) and a parotid biopsy several rheumatic diseases such as Sjoegren's syndrome, IgG4-related disease and sarcoidosis were ruled out and, considering a very high titre of IgE, Kimura's disease was diagnosed. The case and a short review of the literature are presented.

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.

Kimura Disease: A case report with bilateral upper lid masses and a right parotid mass.

A 38-year-old male presented with bilateral upper eyelid masses and a right parotid mass. Nineteen years before the consultation, the patient experienced swelling at both upper eyelids. Formerly diagnosed by an ophthalmologist, corticosteroid, an anti-inflammatory medication, was prescribed for the patient, which resulted in partial resolution of the lid swelling. However, he was lost to follow-up, and progressive enlargement of the lids was observed with the development of a right parotid mass. The visual acuity of the patient was classified as counting fingers, three feet bilaterally. A detailed examination was difficult owing to the obstructive nature of the masses. However, ocular structures appeared intact. An excisional biopsy with a reconstruction of bilateral upper lids was performed. Histopathologic findings revealed a lymphoproliferative lesion suggestive of Kimura disease (KD). The patient was subsequently referred to rheumatology for management, where they started a regimen of oral steroids and antihistamines.

[Kimura's disease. (Clinical case study)]. / Bolezn' Kimury. (Sobstvennoe nablyudenie).

The author presents his own observation of a patient with Kimura's disease (CD), which is a rather rare chronic inflammatory disease of soft tissues. The results of clinical examination and surgical treatment of this disease are presented. It is emphasized that for the diagnosis of CD, first of all, an excision biopsy of the lesion is necessary, as well as the presence of eosinophilia in the patient, an increased level of immunoglobulin E (IgE) in peripheral blood, an increase in regional lymph nodes.

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.

Bilateral Posterior Auricular Masses: A Case of Kimura's Disease.

Kimura's disease (KD) is currently considered a rare chronic inflammatory disease of unknown etiology. It is more common in the Asian population, especially in young and middle-aged men, and can involve the lymph nodes, salivary glands, and subcutaneous tissues. It has been reported in adults and children, and is often accompanied by elevated peripheral blood eosinophils and elevated serum immunoglobulin E (IgE). Herein, we report a case of KD in a 46-year-old man with bilateral masses behind the ears since childhood that had gradually enlarged over 40 years. The patient's peripheral blood eosinophils were elevated, and interestingly, homocysteine levels were also elevated. After surgical resection of the bilateral posterior auricular masses, follow-up over 5 years indicated good recovery and no signs of recurrence.

Eosinophilic interstitial nephritis and cardiac insufficiency in Kimura's disease: a case report.

BACKGROUND: Kimura's disease (KD) is a rare chronic inflammatory disease and the etiology remains uncharacterized. The typical manifestations are painless lymph node or subcutaneous masses. There is currently no report of prominent renal interstitial injury and cardiac insufficiency in KD. CASE PRESENTATION: A 45-year-old man was referred to our hospital with dark urine, subcutaneous masses in forehead and right retroauricular, multiple lymphadenopathy and unexplained cardiac insufficiency. Renal biopsy demonstrated eosinophilic interstitial nephritis. Laboratory tests revealed eosinophilia and a high level of serum IgE. A biopsy of cervical lymph node was performed and KD was diagnosed. Treatment with oral prednisone resulted in a decrease of eosinophil, serum IgE, improvement of cardiac function, and regression of the subcutaneous mass. CONCLUSIONS: We describe an extremely rare KD case presenting with eosinophilic interstitial nephritis, cardiac insufficiency and significant response to prednisone. The clinicians should improve the disease awareness and find optimal treatment.

A comparative study of Kimura's disease and IgG4-related disease: similarities, differences and overlapping features.

AIMS: To compare the fibroinflammatory diseases Kimura's disease (KD) and immunoglobulin (Ig)G4-related disease (IgG4RD) and to explore their possible relationship. METHODS AND RESULTS: Forty-six cases of KD and 29 IgG4RD from our institution diagnosed from 2011 to 2020 were studied. They were compared with each other on clinical, pathological and immunohistological features. There were similar clinical features, except that IgG4RD affected an older patient population, with more frequent salivary gland involvement and KD affected head and neck lymph nodes, and showed blood eosinophilia more frequently than IgG4RD. IgG4RD exhibited frequent storiform fibrosis and obliterative phlebitis, while KD showed more frequent tissue eosinophilia, eosinophilic abscess, germinal centre eosinophilic deposit and vascularisation. Twenty to 30% of KD had more than 50 IgG4+ plasma cells (PC) per high-power field (HPF) and IgG4/IgG+ PC ratio exceeding 40%. These parameters, however, occurred in 100% of IgG4RD. Significantly more KD had >10 IgE+ PC/HPF and lymphoid germinal centre IgE reticular staining compared to IgG4RD. All these histological and immunohistological features are overlapping in the two diseases, although they differed with statistical significance. CONCLUSION: Our study confirmed that there is significant overlap in clinical, pathological and immunohistological features between KD and IgG4RD. It is important to recognise these overlapping features, and correlation with a clinicopathological picture is required in differential diagnoses. The overlapping features also suggest a possible close relationship between KD and IgG4RD, which could represent different facets of a continuous fibroinflammatory disease spectrum.

An Unusual Presentation of Recurrent Childhood Kimura Disease: Ileocecal Involvement.

Kimura disease commonly presents as an isolated swelling over the head and neck region. Intestinal involvement by Kimura disease in children is uncommonly reported. We report a 10-year-old boy who had presented with an ileocaecal mass with peripheral blood eosinophilia and elevated immunoglobulin E levels. The histopathologic examination from the ileocaecal mass was suggestive of Kimura disease. He had 2 recurrences once in the left axillary region and once in the bilateral cervical region. Ileocaecal involvement in Kimura disease is an uncommon presentation in childhood. Careful evaluation of complete blood count is critical in making diagnosis and avoiding unnecessary invasive procedures.

Kimura Disease Presenting with Buccal Mass: A Case Report and Literature Review.

A 52-year-old man with a 2-year history of left buccal swelling was admitted to our department. An elastic hard oral mass was palpated under the intact buccal mucosa. A CT scan with enhancement revealed a solid mass measuring 2.0 × 1.5 × 1.3 cm between the left masseter muscle and the maxilla. Laboratory examination showed elevated peripheral blood eosinophil count of 1070/µL (12.3%) and serum immunoglobulin (Ig)E level of 1374 IU/mL. Histologic examination of transorally excised mass revealed lymphoid follicular hyperplasia with reactive germinal centers and eosinophilic infiltration with eosinophilic micro-abscesses in the germinal centers. Abundant IgE deposition in a reticular fashion was observed in the germinal centers and c-kit positive mast cells was observed in the paracortical area in the excised mass. The patient was diagnosed with Kimura disease (KD) and treated with oral prednisolone, tapering from 10 mg/day for approximately 8 months. Eosinophil count and serum IgE level decreased to 435/µL (5%) and 520 IU/dL, respectively. He is free from symptoms at the time of this submission. KD, a rare, benign, and chronic inflammatory disorder, occurs predominantly in young male adults in Asia. Patients with KD who presents with buccal mass are relatively rare. Immunohistologic analyses suggested that an allergic reaction played an important role in the etiology of KD in this case.

Is Kimura's disease associated with juvenile temporal arteritis? A case report and literature review of all juvenile temporal arteritis cases.

Both juvenile temporal arteritis (JTA) and Kimura's disease are eosinophilic inflammatory conditions but exhibit different clinical manifestations. Here, we describe a case involving a 40-year-old man who developed JTA secondary to Kimura's disease. Approximately 3 years before admission, masses appeared on both posterior auricles. A biopsy of the right posterior auricle mass led to a diagnosis of Kimura's disease. Approximately 4 months before admission, both masses increased in size, and almost simultaneously, the left temporal artery became distended. Histopathology of a biopsy of the left temporal artery revealed inflammatory findings with marked eosinophil infiltration and significant intimal hyperplasia with stenosis of the vascular lumen, indicating JTA. An analysis of the 48 reported cases of JTA, identified in a literature review, and the present case, revealed that Kimura's disease was detected in 6 cases, all of which involved Asians. In conclusion, this case and the literature review suggest that JTA can be accompanied by another eosinophilic inflammation-based disorder, Kimura's disease, particularly in Asians. This newly highlighted relationship between JTA and Kimura's disease could lead to a better understanding of JTA, which is an extremely rare disease.

Warthin-Finkeldey giant cells-A useful clue for the cytodiagnosis of Kimura disease: A report of three cases.

Kimura disease (KD) is a rare, idiopathic chronic inflammatory disorder that usually presents as unilateral painless lymphadenopathy or soft tissue swelling of the head and neck region in young Asian males. The disease lacks pathognomonic clinical and cytomorphological features and can be mistaken for many reactive and malignant conditions. We report three cases of KD presenting as bilateral swelling of the head and neck region, and describe the cytomorphological features of each. In the correct clinical setting, a polymorphous lymphoid aspirate with eosinophils and Warthin-Finkeldey giant cells should raise a suspicion of KD and prompt further work up.