持久性发疹性斑疹性毛细血管扩张症1例

患儿男,15岁。颈部及躯干部淡红色斑丘疹7年,伴轻度瘙痒。皮损组织病理示:真皮浅层毛细血管扩张,周围稀疏单一核细胞浸润,Giemsa染色阳性。诊断:持久性发疹性斑疹性毛细血管扩张症。治疗和症识。     

Prominent Telangiectasia Associated with Marked Bleeding in CREST Syndrome

A 64-year-old woman with CREST syndrome developed prominent telangiectases mimicking hereditary hemorrhagic telangiectasia (HHT) of Osler-Rendu-Weber. We have been following her since she first came to us with discrete telangiectatic mats and Raynaud's phenomenon 11 years ago. Telangiectatic lesions have been seen on her larynx and esophagus in addition to commonly affected sites. She has experienced spontaneous epistaxis and marked bleeding from the lesions on her lips, oral mucous membrane, and soles. This case illuminates new aspects of telangiectasia in CREST syndrome.     

Bloom Syndrome in Two Siblings

Abstract: Bloom syndrome (congenital telangiectatic erythema) is a rare autosomal recessive disorder characterized by telangiectasias and photosensitivity, growth deficiency of prenatal onset, variable degrees of immunodeficiency, and increased susceptibility to neoplasms of many sites and types. We are reporting Bloom syndrome in two brothers from Kashmir (India), 8 and 6 years of age, who presented with erythematous rashes on the face, photosensitivity, and growth retardation.     

A Case of CREST Syndrome Associated with Sick Sinus Syndrome

Cardiac manifestations of the CREST syndrome or limited systemic sclerosis (ISSc) are very rare. We report a case of CREST syndrome associated with sick sinus syndrome. Histopathology of cardiac muscle revealed fibrotic changes, suggesting that such changes may be pathogenetically related to CREST syndrome.     

狒狒综合征并发红皮病1例

患者女,47岁。颈部、双侧腋窝、腰骶部及双股红斑肿胀7天,加重伴发热4天。患者接触汞后,上述部位出现红斑,并出现红皮病样表现。血清转氨酶水平升高,蛋白水平降低。尿汞水平正常。经对症治疗1周后,红斑消退,血清转氨酶水平下降。     

Cowden综合征1例

患者女,19岁,口腔、面、四肢丘疹15年,腹痛2周。皮肤科情况:口腔黏膜、舌部、面部及四肢可见散在皮色角化性丘疹,部分呈疣状或乳头瘤样,面部为甚。胃肠镜检查:消化道多发性息肉。组织病理学检查:(胃窦黏膜)增生性息肉伴中度慢性炎症,HP(+);(舌部)鳞状上皮乳头状瘤;(右前臂)表皮角化过度,局部棘层及颗粒层增生。诊断:Cowden综合征。     

Well’s综合征1例

报告1例Well's综合征。患者女,31岁。四肢反复出现皮疹,伴瘙痒、疼痛1年余,加重1月。组织病理示:真皮内大量嗜酸性细胞浸润,并可见火焰现象。诊断为嗜酸性蜂窝织炎(Well's综合征)。     

Kindler's综合征1例

患者女,26岁。全身反复发生水疱、色素异常伴皮肤萎缩26年。双手足皮肤萎缩变薄,全身皮肤弥漫性色素沉着及部分色素脱失,以面部最重,日晒或受热后加重,无明显自觉症状。结合组织病理诊断为Kindler’s综合征。给予多种维生素及防光剂等治疗,在随访中。     

A Case of Laugier-Hunziker Syndrome

Laugier-Hunziker syndrome is a rare idiopathic acquired hyperpigmentation of oral mucosa and lips which is often associated with longitudinal melanonychia. This condition is known to be an entirely benign disease with no systemic manifestations. Herein we report a case of a 54-year-old woman presenting with a 7-year history of asymptomatic oral pigmentation and linear pigmented streaks of several fingernails. Histological examination taken from the mucosal lesion of tongue revealed increased pigmentation at the epithelial basal layer and a few melanophages in the submucosa. The lack of a family history coupled with the absence of any polyps on the endoscopic examinations of upper and lower bowel as well as the clinical features with late onset were all in favor of a diagnosis of Laugier-Hunziker syndrome. The significance of this disease is due to the need to differentiate this disorder from other pigmentary disorders of the oral mucosa, especially Peutz-Jeghers syndrome.     

静脉曲张性骨肥大综合征1例

患儿女,5岁8个月。右下肢结节、静脉曲张5年,跛行4年。皮损主要分布于右下肢,表现为蚯蚓状隆起的曲张静脉、混合状的血管瘤以及右下肢的肥大增长,同时伴皮肤鱼鳞样改变。结合X线和B超检查诊断为静脉曲张性骨肥大综合征。根据患者临床表现选择治疗方案,如曲张浅静脉的结扎、剥脱术,局部血管瘤激光治疗等。     

Peutz-Jeghers综合征1例

1临床资料患者女,18岁,因面部及唇部色素斑18年前来就诊。患者自出生起唇部即有黑褐色斑点,无任何自觉症状,故一直未予诊断治疗,今前来我科要求激光去除。追问病史,近半年以来偶有腹部疼痛,其父有同样的口唇粘膜黑斑。皮肤科查体：患者面部及口唇部可见较多针帽至粟米大小的黑色或褐色的斑点密集分布,以下唇为甚（图1）。     

Marshall-White综合征1例

报告Marshall-White综合征1例,患者男,29岁,因双上肢浅白斑3年来诊。皮肤科检查:双手背、前臂可见大片淡红色斑片,边界不清,其间散在分布边界清晰的淡白色斑。临床诊断为:Marshall-White综合征。     

抗磷脂抗体综合征1例

患者女,71岁。6个月前间断出现双下肢紫癜,7d前双小腿出现红斑和坏死,疼痛剧烈,抗心磷脂抗体IgM明显升高,狼疮抗凝物(LAC)和抗β2-糖蛋白I(β2-GPⅠ)均(+),皮损组织病理示:小血管内血栓形成。诊断:抗磷脂抗体综合征。予抗凝治疗3周后患者皮损明显好转。提示临床医师对此类坏死皮损应考虑到本病的可能性,及时予以抗凝治疗。     

Wiskott-Aldrich综合征1例

患儿男 ,2个月。因面部反复出现红色小丘疹和紫斑 40天就诊 ,自发病以来经常感冒、咳嗽。患儿的同胞哥哥亦出现过同样病情 ,于生后 5个月时因支气管肺炎、呼吸衰竭死亡。血常规检查血小板计数减少 ,免疫学检查IgG ,IgM ,IgA均高于正常。初步诊断Wiskott Aldrich综合征。给予丙种球蛋白、胸腺肽和抗生素等治疗 ,病情可暂时缓解。随访 4年 ,病情仍反复发作     

Reiter综合征1例

患者男,51岁。左眼红肿8月,颈部疼痛、双足皮疹伴疼痛6月。主要表现为强直性脊柱炎和双踝关节炎,伴有一过性虹膜炎及结膜炎,皮损呈蛎壳样及脓疱样,查HLA-B27阳性,确诊为Reiter综合征。     

Melkersson-Rosenthal综合征1例

患者女,50岁。因唇部反复进行性红肿伴轻微瘙痒半年就诊。皮肤科情况:上唇弥漫性红肿,压之无凹陷,上下唇红斑脱屑。口角略向右侧歪斜,左侧鼻唇沟变浅,吹口哨嘴唇,并向右侧倾斜,伸舌稍向左侧歪斜,舌体肥大,舌面有明显沟纹。上唇皮损病理检查示:真皮浅层可见弥漫性淋巴细胞、少量上皮样细胞和多核巨细胞浸润。诊断:Melkersson-Rosenthal综合征。     

Laugier-Hunziker综合征1例

患者女,55岁。指(趾)甲色素沉着28年,唇部、颊黏膜及齿龈色素沉着21年。无腹部症状。大便潜血试验(-),胃镜及结肠镜检查无异常。皮肤科情况:唇部、颊黏膜及齿龈不规则灰黑色色素沉着斑,部分指(趾)甲纵形棕黑色色素沉着斑。颊黏膜组织病理示:基底层黑素增加,真皮乳头色素失禁和少量噬黑素细胞。诊断:Laugier-Hunziker综合征。