Kimura’s disease: A clinicopathological study of 23 cases

Abstract

IntroductionKimura’s disease (KD) is an uncommon lymphoproliferative fibroinflammatory disorder. Patients present with head and neck subcutaneous nodules with or without lymphadenopathy. Peripheral blood eosinophilia and elevated serum immunoglobulin E (IgE) levels are typical. This study was designed to delineate the clinicopathological features, pattern of care, and disease course of 23 Taiwanese patients with KD.MethodsWe retrospectively analyzed the clinical data of 23 consecutive cases (16 male and 7 female; age at diagnosis: 12–77 years) of KD diagnosed at our institution from 2015 to 2020.ResultsThe median time from presentation to diagnosis was 1 month. Twenty-one patients presented with unilateral or bilateral head and neck masses. The remaining two presented with right flank and right arm lesions, respectively. Peripheral blood eosinophilia was observed in nine, and elevated IgE levels were observed in four. All were diagnosed using either excisional or core-needle biopsy. Seven patients underwent fine needle aspiration without a diagnostic yield. Salivary gland and lymph node involvement was observed in three and seven patients, respectively. Most lesions showed tissue eosinophilia (100%) and florid follicular hyperplasia (78.26%). Three cases had histological KD-IgG4-RD overlap and three had comorbid IgG4-RD were recognized. Thirteen patients underwent surgical resection, one received adjuvant therapy, and two received prednisolone monotherapy.ConclusionKD should be considered in patients with subcutaneous masses, eosinophilia, and elevated IgE levels. Biopsy remains the gold standard of diagnosis. Increased recruitment of IgG4+ plasma cells is a common feature. Consideration of IgG4-RD in all KD patients may be prudent.

Multiomic landscape of immune pathogenesis in Kimura’s disease

“…A retrospective clinicopathological analysis of 23 cases of the Kimura’s disease showed that peripheral blood eosinophilia was observed in nine, elevated IgE levels were observed in four, salivary gland and lymph node involvement was observed in three and seven patients, respectively. 40 Another study summarizing the clinicopathological characteristic of Kimura’s disease in Chinese patients showed that the eosinophil percentages ranged from 5.0–58.7% and the serum IgE levels ranged from 40 to 5000 U/L. 41 These support a heterogeneous pattern of Kimura’s disease in the clinic.…”

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 case of Kimura’s disease is reported because it represents a rare differential diagnosis11–15 to important inflammatory RMD,16 namely primary Sjoegren’s syndrome,17 18 IgG4-related disease19 20 and even eosinophilic granulomatosis with polyangiitis 21. There was no evidence for a hyper IgE-syndrome either 22…”

“…Accordingly, the disease has been described more often in Asians, but it does occur in non-Asians with a similar clinicopathologic presentation. It is a distinctive entity with no known aetiology that reportedly occurs more frequently in Asia where it affects more male patients, and lymph node involvement is common 11–15…”

“…No ultrasound of the salivary glands was performed. After an intensive study of the literature, a diagnosis of Kimura’s disease was made 11–14…”

Kimura Disease: A Rare Case in Vietnamese Woman

“…The condition may be an allergic reaction caused by infection with Candida albicans, parasites, or viruses, or arthropod bites, or may be due to dysregulation of T cell responses in patients with endocrine disorders and/or autoimmune diseases. It could also stem from a Th2 immune response triggering deposition of eosinophils in diseased tissue [18] , [19].…”