Kimura disease is a rare benign chronic inflammatory disorder of the soft tissue with predominant occurrences in young Asian male adults. However, there is limited information of Kimura disease in Southeast Asia. Hence, the clinical characteristics and outcomes of Kimura disease in Malaysia was investigated. Published Kimura disease cases from Malaysia were comprehensively searched in PubMed and Google Scholar up to December 2020 using the keywords “Kimura Disease” and “Malaysia”. Twenty-three papers were identified for review and case series of seven Kimura disease patients from a hospital in Malaysia were descriptively analyzed. A total of 60 cases were obtained from both sources. Eighty-seven percent were men with a male:female ratio of 6.5:1 and majority of the cases were Malays (77.1%). Median age of onset was 22.0 (IQR 12.5-31.5) years, while the median duration before diagnosis of Kimura disease was 2.0 (IQR 0.8-5.0) years. Head and neck region (95%) were the most frequently involved anatomical site. Peripheral eosinophilia was detected in 88.9% of the cases at presentation and renal involvement was observed in four (22.2%) patients. Surgery (57.5%) was the commonest first choice of treatment, followed by a combination of surgery and steroid (29.8%), steroid alone (10.6%), and a combination of steroid and immunosuppressive agent (2.1%). Local recurrences were observed in 28.6% of the cases. In conclusion, a much younger age of disease onset was found among Malaysians. A high recurrence rate of one in every four patients was observed, indicating the need for further evaluations of treatment strategies.
Kimura Disease

ABSTRACT Kimura disease (KD) is a rare chronic inflammatory disorder of unknown aetiology that primarily affects the head and neck region with lymph node involvement. Young to middle-aged adult Asian males are predominantly affected. The most common presentation is painless subcutaneous swelling in the head and neck region, while proptosis or orbital involvement is very rarely reported. KD shares some features with other inflammatory and neoplastic disorders, including lymphoma; thus, investigations to confirm the diagnosis should not be delayed. Systemic corticosteroids are commonly used to treat KD and show an excellent response; however, the optimal treatment is still uncertain, and KD has a high recurrence rate. We describe the case of a patient with KD who presented with proptosis and post-auricular swelling, which responded well to oral prednisolone treatment.
Kimura Disease ; Exophthalmos

Angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura's disease were classified as same disease in the past. Since there are many different clinical and histopathological characteristics that warranted their distinction, they are classified as different disease now. Six cases of Kimura's disease in upper extremity have been reported in Korean literature but ALHE in upper extremity has not been reported yet. We experienced a case of surgically treated ALHE in the upper arm and report this case with review of literature.
Angiolymphoid Hyperplasia with Eosinophilia ; Arm ; Eosinophilia ; Upper Extremity

Angiolymphoid Hyperplasia with Eosinophilia ; pathology ; Child, Preschool ; Humans ; Male

Angiolymphoid Hyperplasia with Eosinophilia ; diagnosis ; pathology ; therapy ; Child ; Humans ; Male

From March 2009 to October 2009, three pediatric patients with parotid tumor were cured. Preoperative physical examination showed regional swelling in parotid area, the surface skin was in moderate reddish purple, the border was vague, and the swelling was inactive. The patients' IgE were significantly increased. B ultrasound examination demonstrated the focus was an isoecho with ringlike dark band around, which was concluded as bull's-eye sign. Magnetic resonance imaging (MRI) examination indicated a cystic mass between the skin and parotid. Preoperative diagnosis was eosinophilichyperplastic lymphogranuloma (Kimura's disease) and the granuloma was excised by operation. Pathological examination revealed the capillary vessel hyperplasia in local tissue with a plenty of eosinophils and lymphocytes infiltrating. The disease was confirmed. Although the disease is rare, the diagnosis still could be made by preoperative physical examination, laboratory and imaging examinations.
Angiolymphoid Hyperplasia with Eosinophilia ; Humans ; Magnetic Resonance Imaging ; Male ; Parotid Gland

Adult ; Angiolymphoid Hyperplasia with Eosinophilia ; Female ; Humans ; Male ; Middle Aged

Angiolymphoid hyperplasia with eosinophilia(ALHE) is a benign, uncommon disorder of unknown etiology, that usually appears as intradermal, subcutaneous or both, red to brown, papules and/or nodules, usually located on the head and neck of young adults and is often associated with peripheral blood eosinophilia. Extracutaneous lesions are rare. We report a 25 year-old male patient who had pruritic dark brown colored, moderately firm nodules and plaques on the left postauricular area, that histopathologically showed features of ALHE. He was treated with triamcinolone acetonide intralesional injection successfully.
Adult ; Angiolymphoid Hyperplasia with Eosinophilia* ; Eosinophilia ; Head ; Humans ; Hyperplasia ; Injections, Intralesional* ; Male ; Neck ; Triamcinolone Acetonide ; Young Adult

Kimura's disease is a rare chronic inflammatory and proliferative condition of unknown etiology. It has been thought to be a part of large spectrum of the entity known as angiolymphoid hyperplasia with eosinophilia (ALHE). However, recently the difference is emphasized between the two entities by some authors. It usually presents subcutaneous or dermal mass in the head and neck region. This condition can be mistaken for a malignant tumor. It is defined pathologically as hyperplastic lymphoid follicles, eosinophilic infiltration and vascular proliferation. Authors have experienced three cases of Kimura's disease occurring in the submandibular, axillary and inguinal regions since 1993. There were two males and one female. Two patients presented peripheral eosinophilia. All patients underwent surgical excision. Two patients were managed with oral prednisone postoperatively.
Angiolymphoid Hyperplasia with Eosinophilia ; Eosinophilia ; Eosinophils ; Female ; Head ; Humans ; Male ; Neck ; Prednisone

Kimura disease (KD) is a chronic angiolymphoid proliferative disorder of soft tissue with eosinophilia and elevated IgE levels, with predilection for head and neck in young oriental men. Renal disease is often associated with it. We describe a 16-year-old male with KD and steroid-responsive minimal change nephrotic syndrome. After surgical resection of cervical tumor and steroid therapy, complete remission of nephrotic syndrome and KD was achieved and there has been no relapse of tumor and nephrotic syndrome with normal IgE levels over 12 months.
Adolescent ; Angiolymphoid Hyperplasia with Eosinophilia* ; Eosinophilia ; Head ; Humans ; Immunoglobulin E ; Male ; Neck ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Recurrence