单发性肥大纠胞瘤1例

患儿，女，4个月，体重7.5kg。因右耳后颈部出现斑丘疹，伴全身反复出现潮红斑、风团4个月于2004年6月就诊。患儿出生时，右侧耳后颈部发现一豆大褐色玉丘疹，渐增大，摩擦刺激后局部肿大潮红，数分钟后肿胀消退。患儿常伴全身发作性红斑，风团和面部红肿，持续数分钟或数小时后渐消退。     

勒-雪病

患者男，８个月。主诉：头皮、面部和躯干暗红色粟粒大丘疹伴发热３个月。现病史：患儿５个月时先于上腹部出现粟粒大暗红色丘疹，很快增多，半个月内波及到头皮、面部及躯干，伴轻度瘙痒。７个月时发热，体温３８～３９℃，腹部逐渐膨胀，双腋下出现肿物。２０d前上腭部发生数个结节及溃疡。近１０d来病情明显加重。发病后患儿进行性消瘦、乏力、食纳差、精神不振。既往史、家族史及个人史：均无特殊。体检：一般情况差，慢性病容，T３８．５℃，Bp１１．９／９．３kPa（１mmHg＝０．１３３kPa），R３８次／min，P１２０次／min。颈及双…     

CLOVES综合征1例

患儿男，4岁3个月，因躯干紫红斑、青色包块伴双足畸形4年3个月于2020年8月4日入院。出生时左髂部及右腰外侧见2处皮肤红斑伴皮肤肥厚，右侧颜面部肿胀，右耳肿大，双足异常；7月龄时在外院就诊，考虑“巨趾症”，予双足多趾、巨趾修复术……     

掌跖角化-牙周破坏综合征合并先天性眼病一例

患儿女，5岁，因四肢丘疹、斑疹、斑片伴瘙痒5年就诊。患儿出生后4个月，双手、双足出现散在针尖至米粒大小红色丘疹，部分丘疹内含清亮液体，瘙痒明显，无疼痛不适，双手足皮疹逐渐融合成片，有皮屑。时好时坏……     

皮肤色素失禁症合并双眼视网膜病变1例

患儿女,1岁7个月。患儿四肢、躯干泛发性皮疹1年3个月。患儿出生即全身皮肤干燥;3个月时,躯干、四肢曾起过水疱;1岁时,出现疣状丘疹和斑片,呈涡轮状排列;4个月前,患儿因"视物不见1个月"到本院眼科就诊,诊断为"双眼视网膜病变"。1个月前,来本院皮肤科门诊就诊,诊断为皮肤色素失禁症合并双眼视网膜病变。目前仍在随访中。     

儿童胫前毛母质瘤

患儿女,4岁。因左下肢起疙瘩3年伴疼痛2个月来诊。患儿1岁时家长发现左下肢伸侧有一绿豆大小结节,触压患儿无特殊不适。近2年结节较前不断增大,近2个月来,患儿在穿衣服或睡觉碰到皮损时,有疼痛感……     

自发突变致先天性厚甲症-K6a

患儿女，4个月。因双手指甲增厚4个月就诊。患儿出生后3 d即开始出现双手指甲增厚、变黄；4个月龄时，双手足20甲均增厚，呈爪状；随访至8个月龄时，双手指甲下充满硬性角质样物质，呈楔形增厚，右手食指、左足拇趾掌侧出现角化；基因检测：KRT6A基因第7外显子存在一个杂合突变c.1390A>C(p.T464P)，患儿父母及其他家属均无相关症状，且其父母基因检测回报未发现该突变位点，结合本患儿临床表现和基因结果，诊断先天性厚甲症（PC-K6a）。     

神经纤维瘤病并发复发性幼年黄色肉芽肿1例

患儿女，出生时多个咖啡斑，生后10月，头面、躯干出现黄色丘疹，4个月后自发消退，7岁时，左下肢再次出现黄色丘疹，组织病理示真皮内以组织，细胞为主的肉芽肿性浸润，并见异物巨细胞及Touton巨细胞，患儿生父患神经纤维瘤病。     

肠病肢端皮炎一例

患儿男，6.5个月。因全身皮疹，腹泻4个月，加重伴发热1周入院。患儿自生后约2个月时臀部、四肢末端无明显诱因反复出现片状鲜红斑，继而糜粒、渗出、结痂；腹泻4～10次/d，黄稀，呈蛋花样，有酸臭味。多次在当地诊为湿疹，外用皮炎平，百多帮等，皮损虽可缓解，皮疹面积扩大，且面积渐增大，3个月前出现脱发。入院前1周，皮疹面积扩大，眼睑缘、口唇糜烂，     

色素性荨麻疹1例

1 临床资料 患儿女，2岁8个月。因全身褐斑，伴瘙痒及红斑、风团2年半就诊。患儿3个月时无明显诱因胸背部开始出现淡褐色斑片，偶感瘙痒。2年来斑片渐累及面部、四肢、颜色逐渐加深，哭闹或情绪激动时在褐斑上出现红斑、风团，短期内可自行消退，但斑片不退。曾至多家诊所、医院诊治，均未见明显好转，为求进一步诊治，遂至本科就诊。患儿发病以来无发热、呕吐、晕厥。     

Dermatology in private practice in France in 2000

OBJECTIVES: Data regarding French dermatological practice are scarce. Our objective was to identify the skin disorders most commonly diagnosed by office-based dermatologists. We also documented the severity of these skin disorders, as reflected by the repercussions on patient's everyday life, and the way physicians managed patients. DESIGN: We carried out a one-day survey of visits to a randomly selected sample of 900 French office-based dermatologists. The randomization was stratified according to the five French different dialing area codes. RESULTS: Office-based dermatologists saw 6411 patients with 7839 skin disorders during the survey. The daily number of visits to French dermatologists was estimated at 47 000 and the annual number between 12 and 14 millions. Office-based dermatologists mostly managed warts, acne, nevus, dermatitis, malignancies and pre-malignancies, fungal infection and psoriasis. Repercussions on patients'everyday life were assessed by physicians as important or very important in 28 p. 100 of cases. Half of the patients received topical treatment, 20.5 p. 100 a systemic drug and 40 p. 100 a minor surgical procedure (including cryotherapy). CONCLUSION: Although dermatologists frequently see benign skin disorders such as warts or nevus, more severe diseases represent an important part of their activity.     

Pentoxifylline in reactions in leprosy

A study was conducted to assess the response of reactions in leprosy to pentoxifylline therapy. Ten cases were studied; 8 cases had type 2 reaction and 2 cases had type 1 reaction. Pentoxifylline was given orally 400 mg three times daily. In patients with type 2 reaction, good response was observed within one week. There was near complete regression of ENL lesions within one month. Cases with type 1 reaction did not respond to pentoxifylline.     

Leukocyte migration in vivo and in vitro in patients with psoriasis

Leukocyte migration in vivo was studied with a skin chamber technique in 21 patients with active psoriasis vulgaris and 18 with cleared psoriasis vulgaris. Measuring over 24 h, no difference was found between healthy volunteers and most patients with active psoriasis, although a subgroup of patients with long-lasting relapses showed subnormal migration values. In patients with cleared psoriasis on the other hand the in vivo leukocyte migration values were increased. In addition, leukocyte migration in vitro under agarose was studied, but no difference was found between healthy controls and patients with psoriasis, active or cleared.     

Decrease in neutrophils observed in vivo in psoriatics after PUVA therapy

Summary In 56 patients leucocyte and differential counts were done before and at weekly intervals during PUVA treatment of chronic recalcitrant psoriasis. A statistical significant (P<0.01) decrease in the percentage of neutrophils was observed during the first week of the PUVA therapy. This observation could be closely related to the clinical clearing of psoriasis (P=0.02).The effect of PUVA therapy in psoriasis may be due to a decrease in the number of immunocompetent neutrophils demonstrated in psoriatic lesions.