儿童银屑病——245例的短期研究和观察

在哥本哈根芬森研究所,作者对245例确诊的儿童银屑病患者用32种指标进行分析。在此研究中女孩多于男孩(64:36)。平均发病年龄是8.1岁,女孩是7.8岁,男孩是8.5岁。三个最早的病例在生后第7天,14天和6个月发病,每个婴儿有一个人数众多的银屑病家庭史。14例在2岁以前发病,47例在2～5岁之间发病。全部病例的59％有阳性家庭史。     

手足口病36例临床分析

1.临床资料 36例患儿均为2009年2-8月本科门诊病例,男22例,女14例.年龄6个月～8岁,平均2.6岁,其中3岁以下30例,占83.0%.2～4月发病6例,5～7月28例,8月2例;幼托儿童25例,散居儿童11例,有明确手足口病接触史者28例.     

皮肤病综合征

20 0 4 1191　葡萄球菌烫伤样皮肤综合征2 7例临床分析/于中蛟(承德医学院附院皮肤科)…∥临床皮肤科杂志.- 2 0 0 3,32 (10 ) .- 577分析结果显示,患儿均为7岁以下儿童。2 7例均有典型皮损,18例发病前有明显诱因,5例患儿的皮肤疖肿或脓疱处培养出金黄色葡萄球菌,10例咽拭子培养有6例阳性,均为金黄色葡萄球菌,15例作血细菌培养均为阴性。全部病例经6～15天治疗均痊愈。提示该病好发于7岁以下儿童,发病诱因多为感染,皮损主要由毒血症引起,早期采用足量有效抗生素是治疗成功的关键。(汤亚娥)2 0 0 4 1192　葡萄球菌烫伤样皮肤综合征2 3例临床…     

自身红细胞敏感性紫癜的同胞姐妹病例报告

自身红细胞敏感性紫癜又称Gardner-Diamond综合征、疼痛瘀斑综合征、精神性紫癜等。1979年后国内有病例报导。本病是否与遗传有关,未见记载。本文报告姐妹二人发病的病例。例一,女,26岁,农民。主诉双下肢反复发生疼痛性瘀斑一年。一年前患者第二次人工流产后,阴道流血半个月。从此常感乏力及下腹坠胀。接着在一次精神创伤后遂有失眠、多梦、抑郁,又有阴道流血,同时下肢出现多处紫红色斑,有疼痛。一星期后斑     

Ramsay-Hunt综合征12例报告

1　病例资料1 1　一般资料　作者自1995年3月～2 0 0 3年3月收集Ram say-Hunt综合征病人12例,其中男5例,女7例,年龄2 8～85岁,平均为6 5岁。如以绝对年龄5 0岁为限,则5 0岁以上者8例,占6 6 7%。发病后就诊时间,除1例为左面肌持续麻痹外,就诊时间为3～10天。1 2　临床表现发病部位　10例位于左侧,两例位于右侧。病损沿面神经,听神经分布,个别病例为三叉神经受累。表现为患侧耳道疼痛,面肌麻痹和集簇分布疱疹。其中两例伴有外耳道,颊黏膜,上腭及舌部疱疹。3例水疱融和大片坏死,两例上睑下垂,两例有舌麻木,味觉减退及吞咽困难。70岁以上的3例…     

无环鸟苷加干扰素周期法治疗复发性生殖器疱疹

生殖疱疹是一种临床上非常难治愈的性病 ,主要由单纯疱疹Ⅱ型病毒 (ＨＳＶ -Ⅱ )引起 ,近几年来 ,随着社会的开放 ,本病的发生逐年增加 ,它主要的特点为周期发作 ,一般为 1～ 4个月 ,每次发作约 8～ 12天。我科门诊近 5年来对196例病人进行了治疗 ,取得了满意效果。报告如下 :1　病例与方法1 1　病例选择　治疗组共有门诊病人 116例 ,男 82例 ,女34例 ,年龄 19～ 5 1岁 ,平均 2 7 5岁 ,病程 3个月～ 4年 ,平均 13个月。复发次数 ,2～ 7次 /年 ,人均复发 4 2 0次 /年。另有对照组 80例 ,男 6 1例 ,女 19例 ,年龄 2 0～ 5 3岁 ,平均2 9 2岁 ,…     

皮肤僵硬综合征五例分析

目的 加深对皮肤僵硬综合征临床及组织病理特点的认识.方法 回顾性分析5例皮肤僵硬综合征患者的临床表现、组织病理学特点和治疗反应.结果 5例患儿发病年龄3个月至4岁,就诊年龄5～9岁,病程2～6年.典型皮疹为一侧下肢及腹部、臀部皮肤木板样坚硬质地,表面毳毛增多,受累关节活动受限.组织病理示真皮内胶原纤维增生,伴有黏多糖沉积.药物治疗的疗效不明显.结论 皮肤僵硬综合征于出生后或婴幼儿早期发病,以一侧肢体受累为主,可累及对侧,需与硬皮病等鉴别,临床尚缺乏有效的治疗手段.     

老年性皮肤病

972772 92例中老年带状疱疹临床分析/宋国刚…∥临床皮肤科杂志.-1997,26(2).-100～10292例中男50例.女42例;中年组(45～59岁)40例,占43.5%,老年组(60岁以上)52例.占56.5%;夏季发病者32例,秋季发病者42例,共74例、占80.4%;神经痛后发疹者85例,占92.4%,神经痛与皮疹同时出现者5例,占5.4%;先有皮疹后有神经痛者2例,占2.2%;老年组患者多在4～6周,中年组多在3～5周,8周以上者均为老年组患者,后遗神经痛者21.例,占22. 8%,持续时间多在3个月以内,最长者持续1年8个月.合并病毒性光膜炎8例.溃疡性角膜炎2例,大疱性鼓膜炎1例,血膜睫状体炎1例.面瘫2例,Ramsay-Hunt综合征1例,病毒性脑炎和脑梗塞1例.表2参5(孙泑秦)     

母细胞性浆细胞样树突细胞肿瘤二例

例1,女,24岁,面部淤青色斑块5个月;例2,男,77岁,全身散发结节、斑块5个月,有"银屑病"病史18年。组织病理真皮内均可见弥漫性母细胞样细胞单一性浸润,与表皮有无浸润带形成;免疫组化均示CD123(+)、CD56(+)、CD4(+)、CD3(-)、CD20(-);诊断为母细胞性浆细胞样树突细胞肿瘤。例1于发病第16个月死亡;例2失访。     

2000-2021年甘肃省新发麻风病例流行病学特征分析

目的:分析2000-2021年甘肃新发现麻风病例流行病学特征与规律，为麻风防控策略的制定提供依据。方法:描述性分析2000-2021甘肃省在全国麻风防治管理信息系统中上报的新发现麻风病例资料。结果:2000-2021甘肃麻风新发病例，年发现率从0.056/10⁵降至0.008/10⁵,年患病率从0.17/10⁵降至0.06/10⁵。共报告新发病例133例，其中男87例，女46例。复发病例共8例，儿童病例4例。2级畸残42例(2级畸残比31.6%),平均延迟期47.70个月。早期发现占12.78%,发现方式以自报和报病为主(80例，占60%),通过接触者检查发现占6.02%,传染源来自家内的病例占31.58%,传染来源不明的占64.66%。平均发病年龄(40.65±13.84)岁，新发病例均为本土病例。全省14个市州有8个市(州)有新病例报告，分布在31个县(区、市)。陇南市报告的病例数占甘肃省报告病例总数的60%,平凉、庆阳、甘南3个市(州)报告病例数占甘肃病例总数的28.6%,病例以散发为主。结论...     

X-Linked ocular albinism; Nettleship-Falls ocular albinism

A 39-year-old man with foveal hypoplasia, nystagmus, and decreased visual acuity was found to have multiple, cutaneous, hypopigmented macules. Macromelanosomes were demonstrated in normal skin on histopathologic examination. The patient's constellation of findings along with a strong X-linked inheritance pattern in family members led to the diagnosis of X-linked ocular albinism, which is an uncommon condition that is characterized by congenital nystagmus, iris translucency, hypopigmentation of the ocular fundus, strabismus, foveal hypoplasia, photophobia, and impaired vision.     

Werner's syndrome: seven cases in one family.

7 cases of Werner's syndrome in one family of northern Sardinia (the female : male ratio being 4:3) are reported. A 9-year-old girl affected with Cooley's anemia is reported too. The typical complete pattern of the syndrome was observed in patients in the fourth decade of their lives, whereas in the two youngest ones, some features were missing. 1 patient died of gastric carcinoma, 1 of cachexia. Consanguinity was established in two generations. The genealogical tree suggests an autosomal recessive mode of inheritance. Genealogical, clinical, biochemical, and histopathological studies were performed. As far as we know, this is the largest number of patients with Werner's syndrome reported in one family.     

Hereditary leiomyomatosis and renal cell carcinoma syndrome: a case report

The simultaneous occurrence of multiple cutaneous leiomyomas, uterine leiomyomatosis, and renal cancer is described as a cancer syndrome with an autosomal dominant pattern of inheritance. We report a 79-year-old man who presented with multiple hyperkeratotic, erythematous nodules on his right leg with a histological diagnosis of pilar leiomyoma. In a review of systems, gross hematuria, weight loss, and bone pain were noted. His pathologic diagnosis was determined to be metastatic papillary renal cell carcinoma. A family history revealed that his sister had a hysterectomy for uterine leiomyomas. The findings in this case can be attributed to hereditary leiomyomatosis and renal cell carcinoma syndrome.     

Linear Darier disease

Darier disease is a genodermatosis with a pattern of autosomal dominant inheritance, which occasionally presents by following a linear or segmental pattern. We present the case of a 22-year-old woman who had presented for three years with small, linearly distributed, yellowish-brown papules on the right lower extremity, with no other skin, mucous membrane or appendage involvement. She had no personal or family history of any interest. Histologically, suprabasal acantholysis and abundant dyskeratotic cells were seen. All of this data led to the diagnosis of linear Darier disease.     

Multiple unilateral skin tumors suggest type 1 segmental manifestation of familial syringoma

An 18-year-old man presented multiple asymptomatic reddish-brown papules with a segmental distribution pattern confined to the left side of the trunk. These lesions had arisen two years before while the rest of the integument was unaffected. His further medical and family history was unremarkable. Histopathology revealed the characteristic features of syringoma. Since familial occurrence of syringoma with autosomal dominant inheritance has been described previously, we propose that the clinical phenotype observed in this patient reflects a type 1 segmental manifestation of familial syringoma and, thus, a cutaneous mosaicism.     

286个白癜风家系遗传方式分析

目的　研究浙江地区白癜风患者的家系遗传特征。方法　以先证者为核心 ,回顾性调查其家系成员白癜风患病情况 ,填写调查表 ,对有家族史者绘制家系图谱。结果　在 2 473例白癜风先症者中 ,有遗传家族史者 2 86例 ,遗传率 11.6%。 2 86个家系中绝大多数均符合常染色体显性遗传特点 ,个别符合常染色体隐性遗传 ,未见性联遗传模式。结论　白癜风的家系遗传以常染色体显性遗传为主。     

Greither's disease

A 14-year-old girl with diffuse palmoplantar keratoderma with hyperhidrosis and progressive extension of keratoderma to the dorsum of the hands and feet is reported. The inheritance pattern was autosomal dominant.     

Congenital familial acral vitiligo

A 22-year-old female presented with vitiliginous lesions on her hands and feet since birth. Similar lesions at similar sites were present in 4 other family members. The trait appeared to have autosomal dominant inheritance with variable penetrance.     

Atrichia with papular lesions

We report a case of atrichia with papular lesions in association with common variable immunodeficiency in an 11-year-old boy. There were identical findings in the patient's father. This rare variant of ectodermal dysplasia typically presents with shedding of normal fetal hair within the first three months. Eyelashes are typically spared. Numerous keratin-filled follicular cysts develop over extensive areas of the skin, usually between the age of 2 and 26 years. These lesions are most numerous on the face, neck, scalp, and extremities. Teeth and nails are entirely normal in these patients. The mode of inheritance of atrichia with papular lesions is uncertain. Our case suggests an autosomal-dominant inheritance pattern, while previous reports have shown autosomal recessive inheritance pattern.     

Familial comedones. Evidence for autosomal dominant inheritance.

Thirteen members of a family had multiple comedones. Three other relatives were also ascertained to be affected. This peculiar dermatosis affected both sexes and was present in one individual as young as 10 years of age. The lesions were more numerous in male family members, increasing in number with age. The pedigree showed a definite pattern of autosomal dominant inheritance.