Clinical analysis of Kimura's disease in 24 cases from China.

BACKGROUND: We reviewed details of Chinese Kimura's disease (KD) cases. A full clinical analysis was subsequently performed to improve the accuracy of clinical diagnosis and treatment of KD. METHODS: A total of 24 patients with pathologically confirmed KD treated between March 2008 and March 2018 were reviewed retrospectively for clinical and histopathological analysis. RESULTS: In the 24 KD cases, 20 were male and 4 were female with the age of onset ranging from 5 to 65 years. Lesion diameter ranged from 0.6 cm to 7 cm with unilateral involvement being more popular (79%). Imaging examination had a high detection rate for KD involving the parotid gland and subcutaneous but had low specificity. Microscopic analysis indicated that KD mainly involved subcutaneous soft tissue and lymph nodes. The prominent feature of lymphoid tissue was germinal center hyperplasia surrounded by several lobules associated with hyperplastic vascular structures. Out of the 24 patients, 11 experienced recurrence of disease after treatment (surgical resection: 46.2%, surgical resection followed by oral corticosteroids: 71.4% and surgical resection combined with radiotherapy: 0%). CONCLUSIONS: Our analysis revealed clinical, imaging, and histological characteristics of KD. A better understanding of the disease will help clinicians reduce misdiagnosis and improve the diagnostic rate upon patient first clinical visit.

The clinicopathological characteristics of Kimura disease in Chinese patients.

INTRODUCTION: Kimura disease (KD) is a rare idiopathic inflammatory disorder of unknown etiology. Unusual presentations of KD might cause diagnostic difficulty or be misdiagnosed as malignancy if clinical suspicion is insufficiently high. Here, we aimed to determine the clinicopathological features of Chinese KD patients to reveal further insights into the natural history and treatment of this disease. METHOD: The clinical data of 46 cases of KD diagnosed at Peking Union Medical College Hospital from January 1980 to December 2018 were analyzed retrospectively through case record review. RESULTS: Of 46 cases, 40 were male and six were female. The age at onset ranged from 2 to 56 years (median 27 years). All patients presented with either single (26.1%) or multi-focal (73.9%) subcutaneous masses. Twenty-nine (63.0%) cases presented with head and neck subcutaneous masses, and 9 cases (19.6%) involved different parts of the body. Parotid, submandibular, and lacrimal gland involvement occurred in 17 (37.0%), 3 (6.5%), and 2 cases (4.3%), respectively. Nephrotic syndrome was present in three cases (6.5%), and thromboembolism was present in five cases (10.9%). During follow-up, thirteen patients (13/28, 46.4%) relapsed over 1-13 years (median 8.5 years). The recurrence rate in patients receiving corticosteroids, surgery, and combined surgery and radiotherapy was 30.8%, 66.7%, and 50.0%, respectively. One patient was diagnosed with T cell lymphoma 1 year after diagnosis of KD. CONCLUSIONS: KD is characterized by subcutaneous masses but it is also a systemic disease. Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.Key Points• Kimura disease (KD) is a rare inflammatory disorder of unknown etiology that is endemic in Asia.• Clinicians must regard and manage KD as a systemic disease.• There is no consensus on optimal treatments and further studies are necessary to improve outcomes.• Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.

Hypervascular Pseudonodular Plaque-Like Ultrasound Morphology in Angiolymphoid Hyperplasia. / Morfología ecográfica en placa pseudonodular hipervascular en hiperplasia angiolinfoide.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular proliferation characterized by solitary or multiple angiomatous lesions. It is most common in young or middle-aged women, and the lesions typically affect the head and neck, showing a particular predilection for the periauricular region. The differential diagnosis in patients with ALHE is broad and includes both benign and malignant conditions. We report on a series of cases of periauricular ALHE in which ultrasound imaging revealed an hypervascular, pseudonodular and plaque-like morphology with clinical and histologic correlations. It also evidenced vascular communication between lesions that appeared to be separate on clinical examination. Familiarity with such ultrasound presentations could help to improve diagnostic accuracy and facilitate disease monitoring in patients with ALHE.

[A cutaneous case of angiolymphoid hyperplasia with eosinophilia: Presentation of a rare entity]. / Un cas cutané d'hyperplasie angiolymphoïde avec éosinophilie : présentation d'une entité rare.

We report the case of a Caucasian 73-year-old woman, without medical history, who is operated of two lesions of the forehead and the scalp measuring 0.4 and 1cm. There is no adenopathy. Histopathological examination revealed a diffuse vascular proliferation of the dermis and the hypodermis composed of small vessels bordered of plump "epthelioid" endothelial cells. There is no mitotic figure. The stroma is slightly fibrous accompanied by a polymorphic inflammatory infiltrate rich in lymphocytes, sometimes arranged in lymphoid follicles, plasma cells and eosinophils. The proposed diagnosis is angiolymphoid hyperplasia with eosinophilia. This is a rare vascular tumor, benign according with the OMS classification and preferentially observed in the skin. We report a cutaneous case, clinical and histopathological data of the reported cases and present the main differential diagnoses.

Angiolymphoid hyperplasia with eosinophilia: report of nine cases.

BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular proliferation characterized by dermal or subcutaneous red or brown papules or nodules, most commonly on the head and neck. OBJECTIVE: The aim of this study was to review the epidemiological and clinical characteristics of ALHE, focusing particularly on the histological and therapeutic features. METHODS: We performed a retrospective study of all cases of ALHE diagnosed in our dermatology and pathology departments between 2004 and 2015. RESULTS: Over 12 years, we collected nine cases of ALHE (0.75 case/year). There were four men and five women. The mean age was 43 years. Lesions presented as erythematous or violaceous papules or nodules in all cases, multiple in five cases, and localized on the head in eight cases or other sites in four cases. The diagnosis of ALHE was clinically suspected in only two cases. The histopathological findings showed an ill-circumscribed, intradermal slightly lobular proliferation of capillary-sized vessels around several central vessels. In all cases, the blood vessels were lined by large endothelial cells. An inflammatory infiltrate around the vessels was formed mainly of lymphocytes and eosinophils with isolated plasma cells and histiocytes. Surgery was the most common treatment in our series. Other local or general treatment has also been used with varying responses. CONCLUSION: Angiolymphoid hyperplasia with eosinophilia is a rare epithelioid vascular tumor with a challenging clinical and histological diagnosis. Despite its benign nature, ALHE causes a therapeutic dilemma.

Epidemiology and treatment of angiolymphoid hyperplasia with eosinophilia (ALHE): A systematic review.

BACKGROUND: Current knowledge of angiolymphoid hyperplasia with eosinophilia (ALHE) derives from retrospective reports and case series, leading to a nonevidence-based treatment approach. OBJECTIVE: We sought to systematically review the literature relating to cutaneous ALHE to estimate its epidemiology and treatment outcomes. METHODS: A literature search of PubMed, EMBASE, Web of Science, and Google Scholar was conducted. Articles detailing cases of histologically confirmed cutaneous ALHE were included. RESULTS: In all, 416 studies were included in the review, representing 908 patients. There was no sex predominance among patients with ALHE. Mean age at presentation was 37.6 years. There was a significant association between presence of multiple lesions and pruritus, along with bleeding. Surgical excision was the most commonly reported treatment for ALHE. Treatment failure was lowest for excision and pulsed dye laser. Mean disease-free survival after excision was 4.2 years. There were higher rates of recurrence postexcision with earlier age of onset, longer duration of disease, multiple lesions, bilateral lesions, pruritus, pain, and bleeding. LIMITATIONS: Potential for publication bias is a limitation. CONCLUSIONS: Surgical excision appears to be the most effective treatment for ALHE, albeit suboptimal. Pulsed dye and other lasers may be effective treatment options. More studies are needed to improve the treatment of ALHE.

Kimura's disease in a young Balkan male.

INTRODUCTION: Kimura's disease is a rare, chronic inflammatory disorder of unknown cause, mainly seen in young Asian men. To our knowledge it has not been reported previously in persons from the Balkan countries. CASE REPORT: We presented a 15-year-old male with Kimura's disease manifested as chronic left neck mass. The diagnosis was based on the histopathological findings of the excised lesion. Peripheral blood eosinophilia and raised serum Immunoglobulin E (IgE) level supported the diagnosis. CONCLUSION: The presented patient confirmed the fact that Kimura's disease could occur in different ethnic groups. Histopathological examination, should be performed prior to making the definitive diagnosis.

Kimura disease: review of the literature.

Kimura disease (KD) is a rare, chronic inflammatory disease of unknown cause and is characterized by painless s.c. swellings and lymphadenopathy commonly affecting the head and neck region. Much therapeutics has been used to treat KD, but is not satisfactory because of frequent relapse. Imatinib has been reported previously to be useful for treatment of hypereosinophilic syndrome and may work by selectively blocking protein-tyrosine kinases, such as platelet-derived growth factor receptor, and c-Kit. We carried out immunohistochemical examination of platelet-derived growth factor receptor-alpha and c-Kit in tissues from patients with KD. The results were positive and suggested that Imatinib might be an effective drug for the treatment of the disease. We have also briefly reviewed the epidemiology, aetiology, clinical manifestations, laboratory and pathological examinations, differential diagnoses, treatment and prognosis of KD in this manuscript.

Skin diseases described in Japan 2004.

During the last century of modern dermatology, more than 30 skin diseases have been described first by physicians from Japan. Many of those conditions were disorders of pigmentation and keratinization, which are quite common in Oriental patients. Since the late 1940s, a number of skin diseases first reported in Japan have gained attention internationally among them being Kimura disease, hypomelanosis of Ito, Kawasaki disease, adult T-cell leukemia/ lymphoma, eosinophilic pustular folliculitis, prurigo pigmentosa, and Ofuji's papuloerythroderma. In this article, we review skin diseases that were first established as distinct entities in Japan, in order to familiarize readers of the Western literature with these conditions.

Kimura's disease (angiolymphoid hyperplasia with eosinophilia) associated with ulcerative colitis.

We treated a 35-year-old Japanese woman who had Kimura's disease associated with ulcerative colitis. Kimura's disease is an uncommon chronic inflammatory disease considered to be of allergic origin based on the presence of eosinophilia and IgE hyperimmunoglobulinemia, and may be a manifestation of a systemic immunologic disturbance. Some immunological complications such as nephrotic syndrome have been reported in association with Kimura's disease, but the present case is the first associated with ulcerative colitis.