皮肤浆细胞增多症1例

皮肤合并系统性浆细胞增多症（cutaneous and systemic plasmacytosis,CSP）是一种以皮肤或多系统成熟浆细胞浸润为特征的良性疾病,最常累及皮肤、淋巴结、骨髓,发病率较低,亚洲人多见,男性发病率稍高,典型的发病年龄为20~55岁。该病的特征为浆细胞多克隆增殖,皮肤浆细胞增多症（cutaneous plasmacytosis,CP）仅累及皮肤,较为罕见,皮损常表现为棕红色斑块,局部外用糖皮质激素或他克莫司、长波紫外线照射有一定疗效。北京大学人民医院皮肤科收治1例皮肤浆细胞增多症,现报道如下。     

T细胞攻击B细胞的靶向作用:外用吡美莫司成功治疗皮肤浆细胞增多症

皮肤浆细胞增多症是一种主要发生于日本患者的罕见皮肤病。本文描述一位75岁白人女性患者,患有面部皮肤浆细胞增多症,伴骨髓受累。与其他皮肤浆细胞增多症相比,患者有低丙种球蛋白血症,尿中游离轻链水平增高。应用主要靶向T细胞的1%吡美莫司外用治疗后,皮损在临床和组织学上几乎得到完全缓解。研究表明由于T细胞的间接介导作用导致治疗上浆细胞诱导消失。T细胞攻击B细胞的靶向作用:外用吡美莫司成功治疗皮肤浆细胞增多症@Hafner C.$Department of Dermatology,University of Regensburg, Franz-Josef-Strauss-Allee 11, DE-93042 Reg…     

成年发作的进行性黏蛋白性组织细胞增多症2例散发病例

进行性黏蛋白性组织细胞增多症是一种局限于皮肤的罕见良性非朗格汉斯细胞组织细胞增生症。文献中已报到过四个家系中的10例全部为女性的患者和1例散发病例。该疾病常发病于儿童期,缓慢进展。作者报道2例散发的无亲缘关系的成年发作的进行性黏蛋白性组织细胞增多症非裔美国女性     

1例皮肤Rosai-Dorfman病发生结晶沉积

Rosai-D orfm an病(RD D)是特殊组织细胞的一种自发反应性增殖,组织细胞内含有大量胞质且通常显示炎性细胞胞质内摄取(共生现象)。这种组织细胞S100蛋白免疫染色阳性且伴典型的淋巴细胞、浆细胞和中性粒细胞浸润。典型临床表现为颈部淋巴结重度增大,组织学表现类似重度窦组织细胞增多病。R D D也可累及淋巴结外,皮肤受累既可作为播散性疾病的一部分,亦可为原发病的结果。作者报道1例罕见的伴结晶沉积的皮肤RDD,一青年男性出现皮肤小结,皮肤活检显示皮肤RDD的典型特征,同时伴发的特征性表现为在许多受损浆细胞、组织细胞胞质内和细胞外…     

浆细胞占50%的反应性浆细胞增多症1例

反应性浆细胞增多症，其骨髓浆细胞一般介于3％到10％，极少超过20％，本次报道1例，初治时骨髓浆细胞达50％。     

真性红细胞增多症并发舞蹈症1例

真性红细胞增多症是一种少见的血液病，其病程中多伴发神经系统症状，而伴发舞蹈症较少见。本院近日收治1例真性红细胞增多症并发舞蹈症，现报道如下。     

僵硬皮肤综合征

僵硬皮肤综合征是一种罕见病,特征为显著的皮肤硬结、轻度的多毛症和关节活动受限,多发生于臀部和股部。已报道多例僵硬皮肤综合征病例,但这些病例常患有多种疾病。作者报道1例中国僵硬皮肤综合征患者,诊断依据患者典型的临床和组织病理学特征。僵硬皮肤综合征@Toyohara J.P.     

多灶性淋巴管内皮肉瘤伴血小板减少症

多灶性淋巴管内皮肉瘤伴血小板减少症是一种非常罕见的疾病。本病表现为弥漫的先天性皮肤和胃肠道血管病变，可以导致严重的胃肠道出血和血小板减少。血管损害的组织病理和免疫组化研究证实为淋巴管内皮细胞起源。治疗效果常不满意。本文报道1例该病的罕见病例，患者为一4周大的婴儿。     

伴有神经系统并发症的真性红细胞增多症115例临床分析

真性红细胞增多症（ＰＶ）是一种少见的、原因未明的、骨髓造血干细胞异常引起的骨髓增生性疾病。临床特点为皮肤粘膜红紫、脾脏肿大、伴血管及神经症状。有报道约９０％的患者可能出现神经系统并发症〔１〕。本文综合我院及国内文献报道伴有神经系统并发症的１１５例真性...     

假性红细胞增多症2例报告

假性红细胞增多症２例报告广东省人民医院（５１００８０）黄梓伦假性红细胞增多症是一种良性红细胞增多，除红细胞、血红蛋白、红细胞压积增高外，其余检查均正常。兹将我院近年来所收治的２例假性红细胞增多症报道如下。１病例介绍例１肖某，男性，５９岁，高干。１９９...     

Primary cutaneous cryptococcosis due to Cryptococcus gattii in Singapore

Cryptococcosis is an invasive fungal infection that occurs worldwide. Infections caused by Cryptococcus (C.) neoformans are commonly associated with immunocompromised patients, while those caused by C. gattii predominantly affect immunocompetent hosts. However, the latter has also been increasingly reported in immunocompromised patients such as those with HIV. Cutaneous involvement by C. gattii in immunocompetent patients is a rare manifestation, with only a few cases reported worldwide. C. gattii infection has rarely been reported in Singapore, with all three reported cases presenting as meningitis in immunocompetent individuals. Its natural habitat is the Eucalyptus tree, which is common in Singapore. We report the case of a 37-year-old migrant worker who had primary cutaneous infection due to C. gattii after sustaining traumatic inoculation.     

Crow-Fukase综合征(附4例报告)

报告4例 Crow-Fukase 综合征。该综合征的主要表现:1.多发性周围神经病变;2.脏器损害;3.内分泌功能障碍;4.骨髓中浆细胞增生或血清 M 蛋白增多:5.多发性骨髓瘤;6.皮肤改变;7.颅内压及脑脊液蛋白增高。本文对其多系统损害的特点、诊断标准、发病机制及治疗进行了讨论。激素治疗     

North American blastomycosis in Central Canada: A review of 36 cases

Experience with 36 cases of North American blastomycosis in Central Canada is reported. Symptoms referable to the respiratory tract predominated, but no uniform clinical pattern for the disease process was evident. Cutaneous and genitourinary tract involvement was present in only a small proportion of patients. The majority of the patients resided in rural areas or were associated with some form of outdoor occupation or activity. No characteristic radiologic appearance could be identified in this series. Amphotericin B administered intravenously is the treatment of choice for this condition. North American blastomycosis is more widespread on this continent than has been previously demonstrated.     

Spontaneous cutaneous endometriosis in Mons Pubis: review of literature

Cutaneous endometriosis is not a very often seen condition and is broadly classified as primary (spontaneous) and secondary. While perineal endometriosis arising in a previous scar has been reported, spontaneous cutaneous endometriosis in perineum is extremely rare and only three cases occurring in mons pubis have been reported in literature. We report a case of 34-year-old lady presented with a swelling in pubic region and associated dull aching pain of 1-year duration with no history of cyclical variation of symptoms. Investigations finally concluded a diagnosis of endometriosis and a Complete excision with clear margins. Clinicians should be aware that a spontaneous endometriosis in the perineum can occur and can have atypical presentation with no increase in size or pain during menstruation.     

Crow—Fukase综合征（附4例报告）

报告4例Crow-Fukase综合征。该综合征的主要表现：1．多发性周围神经病变；2，脏器损害，3，内分泌功能障碍；4，骨髓中浆增生或血清M蛋白增多；5，多发性骨髓瘤；6，皮肤改变；7，颅内压及脑脊液蛋白增高。本文对其系统损害的特点，诊断标准，发病机制及治疗进行了讨论。激素治疗可部分缓解病情。     

Cutaneous endometriosis

INTRODUCTION: Cutaneous or subcutaneous endometriosis is a rare entity that is an often overlooked because of chronic abdominal pain. METHODS: We reviewed the ten cases of cutaneous endometriosis that presented to this hospital over a seven-year period. RESULTS: The mean age of patients at presentation was 36.3 years. All our patients presented with cyclical abdominal pain. There was a considerable delay in the diagnosis and offer of treatment, the mean length of time between onset of symptoms to surgery being 31.7 months (range 1-62 months). The onset was spontaneous in 40 percent of our patients and the rest had scar endometriosis. The patients with spontaneous onset of disease had a more severe pelvic disease compared to those with scar endometriosis. Complete surgical excision was curative but left a large defect requiring polypropylene mesh repair in two patients. CONCLUSION: Cutaneous endometriosis should be suspected in any female presenting with cyclic or non-cyclic pain emanating from a mass in the vicinity of a previous surgical scar, the umbilicus or in the inguinal region. Surgical excision of the cutaneous endometriotic implants can be easily performed and is curative.     

胆囊癌头皮转移1例

胆囊癌的皮肤转移在临床上少见,其头皮转移则更为罕见,目前仅有2例报导。本文中我们记录了一位58岁中国妇女被诊断为胆囊癌头皮转移。据我们所知,这是第一例出现胆囊癌头皮转移但无其他腹腔外转移的病例。     

Amlodipine associated hyperpigmentation

Amlodipine is a potent peripheral and coronary vasodilator with high selectivity for vascular smooth muscle, and is widely used in mild to moderate hypertension, chronic stable angina and vasospastic angina. Its most prevalent side effects are peripheral edema, flushing and headache. Cutaneous adverse reactions associated with amlodipine have been rarely reported. Herein, a male patient is described to develop oral mucosal and cutaneous hyperpigmentation one year after starting amlodipine, which became more noticeable with time. Although cutaneous hyperpigmentation was most prominent on the photoexposed areas, there was no history of previous photosensitivity, pruritus or flushing. To our knowledge, no case of oral and cutaneous hyperpigmentation associated with amlodipine has been formally reported up to date.     

西沙必利对功能性消化不良患者胃电的影响

目的：探讨功能性消化不良（ＦＤ）患者胃电改变及西沙必利对胃电的影响。方法：对３８例ＦＤ患者进行空腹及服西沙必利后６０ｍｉｎ体表胃电图（ＥＧＧ）描记。结果：ＦＤ患者胃电节律紊乱发生在胃体部为４２．１１％，胃窦部为６３．１６％；服西沙必利后６０ｍｉｎ胃体电节律紊乱５０％好转，胃窦部５８．３３％消失。结论：胃动力障碍是ＦＤ的主要原因，胃动力药西沙必利能使胃电节律紊乱好转；对ＦＤ患者可提倡进行ＥＧＧ检查，以指导临床治疗。