木村病一例

<正>临床资料患者,男,33岁。因左耳后出现无痛性肿物3年,于2013年8月26日就诊。3年前,患者左耳后出现一1 cm×2 cm大小的近肤色肿物,偶有轻微瘙痒感,无其他全身症状;肿物缓慢增大,曾于外院检查(具体时间不详),血清嗜酸粒细胞比例显著升高至18.5%,左耳后包块组织病理诊断为"嗜酸粒细胞增多性血管淋巴样增生",未治疗。10年前,患     

Fabry disease. A case report

Fabry disease is an under-recognized X-linked recessive lysosomal storage disorder resulting from the deficient activity of the enzyme alpha-galactosidase A (alpha-Gal A). The first case of Fabry disease in Slovenia was diagnosed in 1991. This 46 year-old male was referred for dermatologic evaluation of a purpura on his abdomen. He was being treated for proteinuria and cardiac symptoms. The diagnosis of angiokeratoma corporis diffusa (Fabry disease) was made clinically and confirmed by demonstration of the deficient leukocyte alpha-Gal A activity. The patient subsequently developed cerebrovascular symptoms, coronary disease, and renal failure, and died from a recurrent myocardial infarction. Family studies identified several other affected males and carrier female relatives with this X-linked recessive disorder. This case illustrates the typical multi-manifestations of this inherited disease which now can be safely and effectively treated by enzyme replacement therapy. Early diagnosis is important for the most effective treatment of this disease.     

Kimura's disease

A 40-year-old white Caucasian man presented with a 6-month history of a 1.5 × 1.5 cm nodular lesion on his left arm. No history of trauma preceeded the lesion and the patient was in good general health.
Dermatologic examination revealed a solitary, red, painless nodule located on the left arm (Fig. 1). Histopathologic examination of punch biopsy revealed lymphoid follicles, some of which had active germinal centers in the deep dermis and subcutaneous tissue. Mixed infiltration of lymphocytes, plasma cells and many eosinophils were present in the interfollicular region. Infiltration of the germinal centers by eosinophils, causing partial destruction, was observed (Fig. 2A). There was also proliferation of thin-walled small vessels and fibrosis in some parts of the interfollicular areas (Fig. 2B). Although Kimura's disease (KD) and angiolymphoid hyperplasia with eosinophilia (ALHE) were considered in the differential diagnosis, it was diagnosed as KD based on these histologic features. Further laboratory examinations were performed. Serum IgE was normal and peripheral eosinophilia was absent.
The lesion regressed after biopsy. Complete healing was achieved with intralesional corticosteroid (triamcinolone acetonide) treatment.     

A case of eosinophilic lymphfolliculosis of the skin (Kimura's disease)

Eosinophilic lymphfolliculosis of the skin (Kimura's disease) in a 31-year-old male is reported. He had two subcutaneous tumors, 10 x 9 x 3 cm and 9 x 7 x 2 cm in size, in the right retroauricular and submaxillary areas respectively. Histopathologic examination of the tumors showed marked infiltration of eosinophils, mast cells and lymphocytes, and angiomatous proliferation of blood vessels from the dermis to the skeletal muscular tissue. In subcutaneous adipose tissue, there were numerous lymphfollicles with germinal centers in which IgE deposition with reticular appearance was observed by immunofluorescent technique. Study of the peripheral blood showed marked eosinophilia and an increase of serum IgE level. These findings suggest the possibility that Type 1 allergic reaction may be involved in the histogenesis of eosinophilic lymphfolliculosis of the skin, although there is no direct evidence for the existence of antigens which react to IgE antibody in the skin lesion.     

Kimura's disease on the scalp

报告1例头皮木村病.患者男,40岁,因右侧头皮结节15年就诊.组织病理检查显示真皮内可见许多反应性淋巴滤泡,淋巴滤泡间区可见大量嗜酸性粒细胞浸润,局部有嗜酸性微脓肿形成.符合木村病的诊断.     

毛囊角化病一例

患者,男,38岁。主因头顶、前额、前胸及后背部皮疹15年,于2011年5月8日来我院就诊。患者15年前无明显诱因头顶及前额出现数个米粒大小的黑褐色丘疹,有瘙痒及轻微刺痛感觉,时重时轻,未经任何治疗。后皮损逐渐增多蔓延至躯干部,颜色加深,夏季瘙痒较严重。     

Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two disease entities in the same patient: case report and review of the literature

BACKGROUND: Much controversy has existed with regard to the relationship between Kimura's disease and angiolymphoid hyperplasia with eosinophilia. They were initially thought to represent the same disease spectrum, but it has now been widely accepted that they are two separate disease entities. To our knowledge, there have been no reports to date describing a case of both Kimura's disease and angiolymphoid hyperplasia with eosinophilia coexisting in the same patient. METHODS: We describe a patient presenting initially with a right postauricular subcutaneous swelling and subsequently developing multiple erythematous facial papules and nodules. The medical literature is reviewed. RESULTS: Clinical features of the right postauricular subcutaneous swelling and multiple erythematous facial papules/nodules suggest Kimura's disease in the former and angiolymphoid hyperplasia with eosinophilia in the latter. Histopathological examinations of these lesions helped to confirm the diagnosis of Kimura's disease and angiolymphoid hyperplasia with eosinophilia, respectively. CONCLUSIONS: Kimura's disease and angiolymphoid hyperplasia with eosinophilia can coexist in the same patient. Coexistence of the two types of lesions in one patient may also be considered evidence that Kimura's disease and angiolymphoid hyperplasia with eosinophilia form a spectrum in one disease.     

Treatment of Kimura's disease with cyclosporine

Kimura's disease is a rare, chronic inflammatory disease of unknown cause. It is characterized by painless subcutaneous swellings and lymphadenopathy, commonly affecting the head and neck region. This is associated with peripheral blood eosinophilia and raised serum IgE. It has distinct histological features of lymphoid follicles, eosinophilic infiltrate, fibrosis and vascular proliferation. The disease usually has a benign, indolent course. Traditionally, therapeutic options have included surgery, radiotherapy and steroids but response has been less than satisfactory. Recently, cyclosporine has been reported to be effective in the treatment of Kimura's disease. In this article, we present a middle-aged Chinese female with Kimura's disease for 20 years and her favourable response to cyclosporine.     

不完全型Reiter病1例

报告一13岁男性患儿 ,临床表现为结膜炎 ,口腔炎、尿道炎、皮肤渗出性粘液性及脓性角化性损害 ,诊断为不完全型Reit er病。经抗生素、糖皮质激素及支持治疗后一般情况好转。本文主要讨论了该病的病因、病理特点、鉴别诊断及其与肿瘤的关系。     

The ultrastructural patterns of mast cell degranulation in Kimura's disease.

BACKGROUND: Although Kimura's disease has been considered an atopic disease because of the association of peripheral blood eosinophilia and elevated serum IgE, the mechanisms of these phenomena are still unknown. OBJECTIVE: It is now established that mast cells are a source of cytokines that are involved in eosinophil recruitment or IgE production. In order to understand the role of mast cells in Kimura's disease, we conducted ultrastructural studies. METHOD: The 45 mast cells obtained from 2 patients with Kimura's disease were classified into two subgroups: S type, with a predominance of scroll or crystal subelements in their granules, and P type, predominantly possessing a particulate or filamentous substructure. RESULTS: We observed numerous P type mast cells with focal losses of granule contents in the absence of granule extrusion in Kimura's disease. CONCLUSION: These results indicate the possibility that slow release of mediators or cytokines from mast cells by piecemeal degranulation may contribute to the pathomechanism of Kimura's disease.     

Penile Crohn''s disease: a case report.

Metastatic Crohn's disease is a rare inflammatory condition characterised by cutaneous granulomatous lesions separated from the affected bowel by normal intact skin. Involvement of the genitalia in Crohn's disease is rare and consists of ulcerated lesions in almost all of the cases reported in the literature. We describe a case of penile involvement in a 27 year old man with a 5 year history of Crohn's disease. Should genital involvement precede the bowel disease, patients may consult the sexually transmitted disease service for this problem and the dermatovenereologists may be the first to formulate the diagnosis.     

Chancriform papule. A case report.

We describe a chancriform papule, a typically chancre like lesion, which was situated well away from the genital area in a patient diagnosed as having secondary syphilis. The diagnosis was confirmed by dark field microscopy, the effectiveness of antisyphilitic treatment, and the presence of a preputial scar.