全身转移性黑素瘤1例

患者女,60岁。头面、躯干、四肢大小不等黑红色结节,无痛痒3年,半年内泛发全身。皮肤科情况:头面、躯干、四肢见大小不等高粱粒至黄豆大小结节,呈皮色、暗红色及黑色。左膝关节上方见一直径约2cm×2cm大小肿块,其上见4～5个大小不等皮色至黑色结节,部分结节表面结痂。皮损组织病理示:真皮内弥漫性肿瘤样细胞浸润,此肿瘤样细胞核大、核仁明显,部分肿瘤样细胞呈巢状排列,肿瘤组织内见坏死。免疫组织化学染色显示肿瘤细胞LCA(-),CK20(-),S100蛋白及HMB45弥漫强阳性,Vimentin弥漫阳性。诊断:黑素瘤。     

痒疹样营养不良型大疱性表皮松解症1例

报告1例痒疹样营养不良型大疱性表皮松解症.患者女,41岁,双小腿丘疹、结节伴瘙痒2年余.皮肤科检查:双小腿伸侧可见密集米粒至花生大小丘疹、结节,周边有色素沉着,部分表面抓痕、糜烂.皮损组织病理:表皮角化过度,真表皮交界处可见裂隙,真皮浅层散在角囊肿,浅层血管周围可见散在淋巴浆细胞及个别嗜酸性粒细胞浸润.免疫荧光:C3(...     

痒疹样营养不良性大疱性表皮松解症1例及家系调查

患者男,30岁。趾甲变形、增厚23年,四肢、躯干丘疹、水疱、结节17年。皮肤科检查:全身多处绿豆至蚕豆大小粉红色扁平丘疹、结节、瘢痕,表面可见糜烂、结痂,趾甲均变黄、变形、增厚。皮肤组织病理:角化过度,表皮萎缩,表皮突消失,表皮下裂隙形成;真皮浅层血管扩张,淋巴细胞浸润,胶原增生,散在表皮囊肿。诊断:痒疹样营养不良性大疱性表皮松解症。家系调查:4代共21例,有8例患病,其中男5例,女3例,符合常染色体显性遗传。     

痒疹样营养不良型大疱性表皮松解症1例

报告1例痒疹样营养不良型大疱性表皮松解症。患者男,35岁。双小腿丘疹、结节伴瘙痒4年余。皮肤科检查:双小腿伸侧皮肤密集米粒至黄豆大暗红色丘疹、结节,部分皮损表面有鳞屑、结痂,无明显水疱,指(趾)甲未见异常。口腔黏膜未见损害。皮损组织病理示:表皮部分增厚,表皮下见裂隙;真皮浅层乳头完整,浅层小血管增多,血管周围个别嗜酸性粒细胞和少量淋巴细胞,毛囊上皮细胞水肿,周围见少量淋巴细胞。直接免疫荧光:IgG、IgM、IgA、C3均(-)。诊断:痒疹样营养不良型大疱性表皮松解症。     

获得性反应性穿通性胶原病1例

患者男,50岁,躯干、四肢丘疹、结节伴痒6个月,加重半月。皮肤科情况:躯干、四肢多发绿豆至黄豆大小红丘疹,表面抓痕、结痂,双肘部散在、暗红色蚕豆大小结节,中等硬度,大部顶端见溃疡,中央有脐凹;皮损组织病理示:HE染色见杯状下陷表皮,内含大量破碎角质、中性粒细胞及变性胶原纤维,见断裂、卷曲的胶原纤维束垂直从表皮穿过;真皮浅层见中性粒细胞、淋巴细胞浸润。Masson染色可见蓝染破碎、变性胶原纤维穿出表皮,穿透部位弹力纤维染色阴性。诊断:获得性反应性穿通性胶原病。     

痒疹样营养不良型大疱性表皮松解症

患者女，46岁。主诉：全身皮肤红色丘疹、水疱伴瘙痒30余年。现病史：患者30余年前无明显诱因双小腿及双前臂皮肤瘙痒，搔抓或轻度碰撞、摩擦后易出现水疱，水疱糜烂结痂痊愈后留有瘢痕样红色丘疹。部分皮损表面出现白色粟粒样丘疹，可挤出小的白色颗粒。病情迁延不愈，皮损渐增多并蔓延至四肢近端及躯干。患者患病期间曾在多家医院诊治，曾诊断为“痒疹”、“结节性痒疹”、“皮肤淀粉样变”等疾病，口服“泼尼松”、“氯雷他定(开瑞坦)”、“西替利嗪”等药物，外用“丙酸氯倍他索霜(金血尔)”、“曲安西龙(去炎松)霜”等药物，疗效不明显，遂来我院就诊。     

混合型汗孔角化症1例

报告1例混合型汗孔角化症。患者男,55岁,躯干、双下肢结节伴痒5年余。体检:面部见多发的绿豆至花生米大小环状斑片;躯干、四肢散在黄豆至蚕豆大小结节,高起皮面,质较硬;会阴、阴囊见数个大小不等、质地较硬的结节或疣状增生性斑块。皮损组织病理检查:表皮角化过度,见角化不全柱,其下有角化不良细胞,颗粒层、棘层增厚,呈银屑病样增生。根据临床及皮肤组织病理,诊断为混合型汗孔角化症。     

播散型血管淋巴样增生伴嗜酸粒细胞增多一例

患者男,３７岁。因面部、四肢、躯干起红色丘疹、结节伴痒１０年,加重２年,于２０００年８月１５日入院。患者１０年前因蚊虫叮咬,面部、双手背、足背、四肢起米粒大小红斑及红色丘疹,皮损散在,微痒。在当地医院就诊,经对症处理皮损可消退,但停药后皮损又复发。以后皮损累及躯干,逐渐增多,出现米粒、黄豆大小丘疹、结节伴痒。最初３年每年４、５月为重,冬季减轻。近７年来皮损逐渐增多,皮损发展呈暗红色、淡褐色,呈黄豆大小丘疹、结节,伴痒,抓破及外伤处结节样皮损增多。在劳累、饮酒及食辣椒后皮损明显加重,经休息、停用刺…     

获得性反应性穿通性胶原病1例

1临床资料患者女,48岁,面部躯干四肢红斑结节溃疡伴痒3个月。患者3个月前无明显诱因颈部出现散在红斑、丘疹、结节,伴瘙痒。病程中皮损逐渐蔓延至面部、躯干、四肢、臀部,多数丘疹中央出现脐凹,中央覆以蜜黄色结痂,脐凹逐渐扩大呈黄豆至分币大小。发病后患者于当地医院按"湿疹"治疗1个月,皮疹无好转,逐渐增多,遂至本院进一步诊治。     

伴BRAF-V600E基因突变的婴儿朗格汉斯组织细胞增生症1例

患儿女,6个月,躯干、四肢肤色丘疹3个月。皮肤科情况:躯干、四肢见多数米粒至绿豆大小的肤色丘疹,部分皮疹中央可见坏死、结痂,伴有散在淡白色素减退斑。皮损组织病理示:真皮乳头层可见密集的上皮样细胞浸润;免疫组织化学示:CD1a(3+),S100(3+),基因检测:BRAF基因外显子15c.1799TA,p.(Va1600G1u)突变检测阳性。诊断:朗格汉斯组织细胞增生症。     

痒疹样营养不良型大疱性表皮松解症一家系调查

报告痒疹样营养不良型大疱性表皮松解症一家系调查结果。该家系共5代34名成员,其中患病者15例(男8例,女7例),属常染色体显性遗传。先证者,女性,18岁。于1岁左右双踝部出现数个水疱,双胫前皮肤在外伤、搔抓后形成圆形或卵圆形丘疹、结节,似米粒至花生米大,暗红色,质地较硬,部分皮损表面有痂壳。双足多个趾甲增厚或脱失。皮损组织病理学表现为多处表皮下裂隙形成,真皮内散在较多的表皮样囊肿,并有少量淋巴细胞浸润。该家系中其他患者的皮损与先证者类似。     

Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis

Malignant atrophic papulosis (MAP), or Degos syndrome, is a rare disorder of unknown etiology. It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules. We report the case of a 42-year-old woman with a history of progressive systemic sclerosis who presented with painful subcutaneous nodules on her abdomen along with chronic atrophic papules on her upper and lower limbs. Biopsy results of both types of lesions revealed vascular thrombi without surrounding inflammation. We briefly review the literature on MAP and its association with various connective tissue diseases. To our knowledge, there have been no previous reports of a patient with the clinical and histologic presentations described here. Although the histologic appearance of the subcutaneous nodules was very similar to that of the atrophic papules, the clinical characteristics of the 2 types of lesions were strikingly different. It is fair to theorize that Degos lesions do not start as atrophic porcelain-white papules but rather evolve from a primary lesion. We hypothesize that these lesions start as painful red nodules and may represent part of the disease spectrum in the evolution of MAP.     

无肌病性皮肌炎一例

患者,女,21岁。面部、躯干、四肢皮肤红斑、丘疹伴脱发6年,皮损基本损害为红斑,双眼睑见明显水肿性紫红斑,其他部位皮损为弥漫性对称性暗紫红色斑,双手可见Gottron丘疹。肌力正常。皮损组织病理检查：表皮轻度角化过度,基底膜增厚,真皮浅层水肿,血管周围单一核细胞浸润。诊断：无肌病性皮肌炎。给予泼尼松联合甲氨蝶呤治疗2个月后皮损明显好转。     

海分枝杆菌感染一例

患者，女，73岁。右上肢皮疹3个月，出疹前半个月有海鱼刺伤史。皮肤科检查：右上肢可见数枚半球形红色丘疹和结节，直径0.3~1.5 cm，沿淋巴管分布。皮损组织病理检查：表皮棘细胞增生，真皮内可见以中性粒细胞为主的混合炎症细胞浸润，偶见多核巨细胞。结核感染T细胞斑点试验(T-SPOT.TB)阳性，病原宏基因组学检测示海分枝杆菌阳性。诊断为海分枝杆菌感染。给予克拉霉素及利福喷丁治疗1个月后好转。     

播散性带状疱疹后肉芽肿性炎型Wolf同位反应一例

【摘要】 患者男,65岁,因右面部红肿20 d,背部、双上肢散在丘疹10 d入院。发病前20 d曾因播散性带状疱疹住院治疗。确诊慢性淋巴细胞白血病3年。皮肤科检查：右面部眼睑以下区域、耳廓及外耳道弥漫性暗红色肿胀性斑块,触之有浸润感,原带状疱疹愈合后遗留散在褐色结痂,其间可见散在凹陷性瘢痕;颈后、背部及双上肢可见散在浸润性淡红色丘疹,绿豆至黄豆大小,表面较光滑。面部皮损组织病理检查：真皮全层及皮下脂肪层可见上皮样细胞和淋巴细胞呈结节状浸润,并含较多多核巨细胞;免疫组化：CD68、CD20、CD79a、CD3、CD2、CD10、CD5、Bcl-2阳性、Ki-67散在阳性,CD23、细胞周期蛋白D1、Bcl-6、多发性骨髓瘤癌基因1、CD21、CD35、髓过氧化物酶均阴性。诊断：播散性带状疱疹后肉芽肿性炎型Wolf同位反应。治疗：静脉滴注甲泼尼龙40 mg/d,皮损逐渐好转消退,随访4年未复发。     

黏液水肿性苔藓2例

报告2例黏液水肿性苔藓。例1.女,16岁。全身起丘疹、结节2个月,以手部结节损害为特征。例2．女,40岁。四肢、躯干丘疹3年,伴面部斑块1年,以眉间隆起的纵向斑块为特征。此2例患者的丘疹、结节组织病理检查均示真皮胶原纤维疏松,成纤维细胞增生,胶原纤维束间阿新蓝染色阳性。     

表皮痣综合征

报告1例并发癫癎的表皮痣综合征.患者女,21岁.出生后3个月颈部、躯干、四肢即出现弥漫分布的黑褐色、疣状角化性丘疹.患者3年前出现癫癎间断发作.家族中有同样疾病患者.皮损组织病理检查示表皮角化过度,基底层黑素增多.     

Congenital self-healing histiocytosis (Hashimoto–Pritzker)

BACKGROUND: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition, initially seen at birth or in the neonatal period, with generalized papules, vesicles, or nodules. Affected infants are otherwise well and the skin lesions tend to involute spontaneously within weeks to months. METHODS: Twelve patients with CSHLCH were seen from 1989 to 1998. RESULTS: Eight patients were girls and four were boys and all presented with lesions at birth which disappeared 1-3 months later. The lesions consisted of numerous brownish-red papules, papulovesicles, crusts, and nodules distributed on the face, limbs, palms, and soles. Two patients had oral mucosal lesions, and one had ulcerated lesions that evolved leaving hypochromic macules. Light microscopy showed a histiocytic infiltrate in the papillary dermis with epidermotrophism. Two cases were studied by electron microscopy: the Langerhans cells showed Birbeck granules and laminated corpus in their cytoplasm. Immunomarking with S100 protein was performed in all 12 patients and was positive. CD1 was also tested in four cases and was positive. CONCLUSIONS: Because CSHLCH is a rare condition, we emphasize that, although it is usually a benign, self-limited entity, careful evaluation for systemic disease must be performed and long-term follow-up must be carried out to detect evidence of relapse or progression of the disease; this is essential when treating these patients.     

Epidermodysplasia verruciformis

We present the case of a 50-year-old Latin American woman who consulted her physician because of recent pruritic lesions on her arms and thighs. During the examination, we observed multiple flat papules on the limbs, as well as hypopigmented macules on the trunk which, according to the patient, began to appear during childhood. A histological study was performed on both types of lesions, and showed some enlarged keratinocytes with light blue cytoplasm in the upper layers of the epidermis. More than 20 types of HPV associated with EV (HPV-EV) have been described. Although it was previously thought that these were specific to EV, new molecular biology techniques have made it possible to isolate HPV-EV sequences in skin diseases, both benign and malignant, with epidermal hyperproliferation in the immunocompetent population.     

Disseminated angiolymphoid hyperplasia with eosinophilia: a case report

A 37-year-old Cantonese man presented with pruritic erythematous papules and nodules on his face, limbs, and trunk that had been present for 10 years and aggravated for 2 years. More nodules were noticed where the skin was scratched and traumatized. The lesions were alleviated temporarily, but they did not subside entirely without therapy. The lesions responded to treatment with intravenous dexamethasone. Histopathology results indicated angiolymphoid hyperplasia with eosinophilia (ALHE), and the patient was diagnosed with disseminated ALHE (DALHE). His lesions ameliorated after treatment with prednisone.