疣状痣并发脂溢性角化1例

患者男,34岁。右侧腹股沟疣状丘疹30年余,伴菜花状突起10年余。皮肤镜示:(疣状丘疹)聚集成簇的棕色大圆环;(菜花状突起)脑回状结构、多发粟粒样囊肿、粉刺样开口。皮损组织病理示:表皮疣状增生,角化过度,可见角囊肿,棘层基底样细胞增生,胞浆中色素颗粒,真皮浅层少量淋巴细胞及组织细胞浸润。诊断:疣状痣,脂溢性角化症。     

表现为糜烂性斑块的蕈样肉芽肿1例

患者男,65岁。周身斑块伴瘙痒6年,糜烂渗出2年。皮肤科情况:面颈部、躯干上部、四肢、腋窝、腹股沟、肛周可见大小不等的红色斑块,斑块表面糜烂渗出,局部可见结痂。皮损组织病理示:表皮增生,轻度水肿,部分表皮糜烂,真皮团块状淋巴细胞浸润,少量嗜酸性粒细胞,部分淋巴细胞侵入表皮。免疫组化示:CD3(+),CD4散在(+),CD8(+),CD20(-),CD30(-),CD79a(-),Ki-67(30%~40%+)。诊断:蕈样肉芽肿(ⅡA期)。     

外阴朗格汉斯细胞组织细胞增生症1例

报告1例外阴朗格汉斯细胞组织细胞增生症。患者女,44岁,外阴大片丘疹、结节、糜烂伴痛痒感1年余,渗液1周。皮损组织病理示:皮肤局部糜烂,底部一致性细胞组织样细胞增生,可见核仁与核沟,未见核分裂。瘤细胞内散在少量中性粒细胞浸润,周围可见簇集状淋巴细胞浸润,嗜酸性粒细胞浸润不明显。免疫组化:S-100(+)、CD1a(+)。诊断:外阴朗格汉斯细胞组织细胞增生症。     

疣状表皮发育不良伴Bowen病1例临床分析

目的：报道1例花斑癣型疣状表皮发育不良伴外阴部鲍温病,并对患者两处皮损的临床特点、病因及相关性进行分析。方法：将腰背部及外阴部皮损的石蜡包埋组织切片提取DNA后,使用凯普HPV37型基因分型试剂盒以及PCR扩增方法进行检测,同时对患者进行治疗。结果：两者试验结果未能体现两处皮损相关性,推测外阴皮损不是疣状表皮发育不良发展而来,为单独起病。患者T淋巴细胞明显降低。采用ALA-光动力治疗外阴皮损,三次光疗后皮损变化不明显,予以局部肿物完整切除术后,随访半年无复发。采用维A酸治疗疣状表皮发育不良皮损,治疗4个月后四肢疣状增生样皮损较前稍许变平,色素脱失斑片部分融合、边界较前模糊。结论：此病例伴发的鲍温病与疣状表皮发育不良无相关性;T淋巴细胞等因素可能在疣状表皮发育不良发病机制中占有作用。     

种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤1例

患者女,17岁。全身反复起丘疹、水疱、坏死、凹陷状瘢痕伴瘙痒、发热15年,四肢起肿块2年。血清抗EBV-IgM(-),抗EBV-IgG(+)。肿块处皮损组织病理示真皮中下层和皮下组织见弥漫性致密的瘤细胞浸润,细胞核呈间变性;免疫组化示CD3(+),浸润的大细胞CD30(+),CD43(+),80%浸润细胞Ki-67(+)。水疱处皮损组织病理示表皮网状变性及多个水疱,真皮和皮下组织可见血管和附属器周围以淋巴细胞为主的、伴少量嗜酸粒细胞浸润,部分浸润细胞呈明显异形性;免疫组化示CD3(+),CD30(-),CD43(+),Ki-67(+)。诊断:种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤。确诊后建议患者转肿瘤科化疗,随访中。     

以外阴湿疹样皮损为首发表现的成人朗格汉斯组织细胞增生症

报告1例以外阴湿疹样皮损为首发表现且侵犯骨骼的成人朗格汉斯组织细胞增生症(LCH)。患者女,43岁。外阴红斑、丘疹、渗出伴瘙痒2年,肛周疣状增生物伴瘙痒1个半月。皮肤科检查:外阴淡红色斑块,表面有糜烂、结痂,伴轻度渗出;肛周可见疣状增生物。肛周皮损组织病理检查:真皮内组织细胞及淋巴细胞浸润。免疫组化检查示S-100蛋白及CD1a均(+)。骶髂关节CT示左侧髂骨膨大、骨质破坏、外侧缘骨皮质增厚,部分骨皮质不连续。诊断:LCH。治疗:给予沙利度胺0.1 g,每日2次口服,皮损明显好转。     

外阴Paget样网状细胞增生症

报告1例外阴Paget样例状细胞增生症．患者女，70岁，外阴红斑，增生，破溃，渗液2年，皮肤科检查见外阴及其周围有一11cm×10cm浸润性暗红斑，边界清楚，边缘略隆起，皮损组织病理检查示表皮下部和真皮上部可见不典型单核细胞浸润，多个异形细胞聚集成巢，甚似Pautrier微脓肿，免疫组化染色结果示CD3、CD43、Ki-67均（＋），CD4、CD8、CD20、CD79α、DC30均（-）。诊断：阴外Paget样网状细胞增生症。[编者按]     

发热型溃疡型急性痘疮样苔藓样糠疹合并肝炎和肺真菌感染1例

患者男,34岁,躯干红斑、丘疹半月余,泛发全身10d。皮损为密集分布的大小不一的红斑、丘疹,部分红斑、丘疹上可见米粒大小的水疱及浅溃疡和暗黑色痂,水疱疱液清,壁厚,尼氏征阴性。躯干见散在色素沉着斑,无明显脓疱、糜烂、渗液。皮损组织病理示:表皮角化不全伴少许炎细胞浸润,表皮内和真皮浅层广泛红细胞外渗,表皮细胞内和细胞间水肿伴变性、坏死,部分棘层松解,表皮和真皮浅层界限模糊不清,真皮浅、中层小血管周围致密淋巴细胞、组织细胞浸润。乙肝表面抗原、乙肝e抗原、乙肝核心抗体三项阳性。诊断:发热型溃疡型急性痘疮样苔藓样糠疹。服用强的松1个月后,患者行支气管镜检查刷片示真菌孢子阳性,痰培养示白色念球菌,予氟康唑胶囊治疗2周后症状好转。     

幼儿泛发性扁平黄瘤1例

患儿女，2岁，头面部黄色斑片1年半，渐累及躯干、外阴。皮肤科情况：头面部、躯干、外阴可见多发大小不一的黄色斑片，约绿豆至豌豆大小，边界清，表面光滑，未见丘疹、斑丘疹、鳞屑、萎缩、糜烂、坏死等。腹部皮损组织病理示：真皮上部可见弥漫泡沫细胞浸润，伴有淋巴细胞浸润。诊断：泛发性扁平黄瘤。     

头面部疣状角化不良瘤并发基底细胞癌一例

报道一例头面部疣状角化不良瘤合并基底细胞癌。患者,女,79岁,因左耳前糜烂、溃疡3年;头顶丘疹2年余入院。皮肤科检查：左耳前约1cm×3cm大小皮肤糜烂面,表面黑色结痂,边缘轻微隆起,少量渗液。头顶部有0.5 cm×0.3 cm大小丘疹,表面粗糙,中央呈脐状凹陷,基底潮红,触之质地坚硬。入院后在基础麻醉加局部浸润麻醉下行左耳前基底细胞癌扩大切除加邻近皮瓣修复术,术中一并完整切除头顶部皮损。术后标本病理检查示：头顶部丘疹：表皮内见两个杯状凹陷,其内充满角质物和大的角化不良细胞,下方颗粒层消失,并见棘层松解现象,真皮浅层见噬色素细胞,可见大量淋巴细胞浸润。左耳前斑块：角化过度,肿瘤团块位于真皮内,部分与表皮相连,由基底样细胞组成,周围栅栏状排列,肿瘤细胞内可见粘液样物质。诊断疣状角化不良瘤合并基底细胞癌。     

淋巴瘤样丘疹病1例

患者男,27岁。躯干、四肢反复发生暗红色丘疹、结节、坏死、结痂4月余,无痛,偶有瘙痒。皮肤科情况:躯干、臀部及四肢暗红色丘疹、结节,部分中央坏死、破溃,结黑褐色厚痂,留有萎缩性瘢痕或色素沉着。皮损组织病理示:真皮结节状淋巴及大组织样细胞浸润,可见多数异型细胞,约占30%,少数细胞呈双核或多核;免疫组化染色示:CD3(+),CD4(+),CD30(+),CD68(+),CD8个别(+),CD20个别(+),Ki-67约10%(+)。诊断:淋巴瘤样丘疹病。     

多脏器累及的亲表皮CD8~+ T细胞淋巴瘤1例

患者男,46岁,全身渗出性红斑、头皮脓肿9月。入院检查发现累及肺、肝脏,皮肤组织病理示:表皮海绵水肿,真皮弥漫淋巴细胞,异型细胞具亲表皮性,免疫组化示:LCA(+),CD8(+),TIA(+),TCR基因重排阳性,诊断:亲表皮CD8+皮肤T细胞淋巴瘤。予以2次CHOP化疗,无效死亡。本病在国内首次报道,具有独特临床病理特点,早期累及皮外,侵袭性高,无有效治疗方法,预后差。     

牛痘样水疱病样原发性皮肤CD8阳性T细胞淋巴瘤一例

一例13岁女孩面部和四肢为主反复出现红斑、丘疹、水疱样皮损9年,皮疹形态与牛痘水疱病相似(hydroa vacciniforme,HV)。在病情急性进展期,伴高热,体温39℃以上。皮肤科检查:面部水肿,面部和四肢较多的萎缩性圆形和椭网形痘疮样瘢痕,散在红斑、丘疹、坏死和结痂;四肢数个水肿性红色斑块,中心有圆形破溃面。组织学上,在真皮的血管和附属器周围,可见大量表达CD8异形淋巴细胞浸润,诊断CD8⁺皮肤T细胞淋巴瘤。糖皮质激素和免疫抑制剂可使病情缓解,随诊至今,无系统受累情况。     

毛囊角化病1例

患者男,38岁。面部及四肢、躯干密集毛囊性丘疹20余年。皮损表现为密集针头及米粒大小褐色毛囊性丘疹,表面油腻性污痂,双侧腹股沟疣状增生。皮损组织病理示:角化过度伴灶状角化不全及角栓形成,棘层增生肥厚,呈疣状增生,表皮上部棘刺松解,可见圆体和谷粒。真皮浅层有少量淋巴细胞为主的炎症浸润。诊断:毛囊角化病。     

Multiple capillary hemangiomas: a distinctive lesion of multicentric Castleman's disease and POEMS syndrome

A diagnosed case of Castleman's disease, proven by biopsy from enlarged inguinal lymph nodes, presented with multiple, asymptomatic, erythematous papules and nodules prevalent since nine years over the trunk and extremities. The lesions had been gradually increasing in number and size. The patient had had plasmacytoma of the lower thoracic vertebra 12 years ago, for which he was adequately treated with chemotherapy and local radiotherapy. Dermatological examination revealed erythematous papules and nodules on the face, trunk, and extremities that were diagnostic of capillary hemangiomas. Histopathology of the erythematous, soft papule was suggestive of capillary hemangioma. Contrast-enhanced computerized tomography of the abdomen and pelvis showed multiple retroperitoneal nodes suggestive of Castleman's disease along with multiple osteolytic lesions in the pelvic girdle and vertebrae. The patient was treated with injection rituximab and is currently under follow-up. We report this case to highlight a rare association between Castleman's disease and POEMS syndrome.     

Langerhans cell histiocytosis in an adult: good response of cutaneous lesions to acitretin

A 57‐year‐old man presented with a 2‐year history of bilateral erosive lesions on the inguinal region, and erythematous, brown and crusted papules over the trunk. Histological examination of one lesion in conjunction with immunohistochemical study and electron microscopy led to the diagnosis of Langerhans’ cell histiocytosis. After a thorough examination, the only associated findings were retroperitoneal fibrosis and hypergonadotrophic hypogonadism with a granulomatous testicular infiltrate. The patient was treated with oral acitretin for 1 year (with a topical corticosteroid for the inguinal lesions), resulting in clearing of the cutaneous lesions. He underwent placement of bilateral double‐J ureteral catheters and was started on hormone replacement therapy. At follow‐up 1 year after treatment with acitretin ceased, the patient remained free of cutaneous lesions and his overall condition, including the retroperitoneal fibrosis, had improved. This case had an uncommon combination of features, with a good response to acitretin.     

亲毛囊性蕈样肉芽肿四例分析

报道4例亲毛囊性蕈样肉芽肿。男3例,女1例,年龄32 ~ 52岁。3例以头面部及颈部受累为主,密集多发性暗红色不规则浸润性斑块、结节、肿块、毛囊性丘疹及痤疮样皮损,躯干、四肢表现为斑片、轻度浸润的斑块、毛囊性丘疹及痤疮样皮损。1例头面部未受累,全身密集毛囊性丘疹。组织病理显示真皮内、血管周围及毛囊周围大量淋巴样细胞为主的团块状浸润,部分细胞异形,浸润细胞移入毛囊上皮是他们共同而显著的特征,而亲表皮现象不明显。其中2例毛囊内充满黏蛋白,阿新蓝染色阳性。免疫组化显示浸润细胞主要为CD4阳性T细胞。对常规用于经典蕈样肉芽肿的治疗反应差,病情难以完全缓解,即使部分缓解后也极易复发。     

Papuloerythroderma

The main macroscopic features of 4 cases presented here are erythroderma-like lesions and solid papules. The former covers extensive areas over entire trunk and extremities, especially extensor surfaces. The latter is observed mainly on flexor surfaces of extremities and around the former. The axillae, inguinal regions, cubital and popliteal fossae, and big furrows on abdomen are spared from these lesions. Rashes appear first as papules and form erythroderma-like lesions through their assembly and confluence. Histologically, both show almost the same findings. Patients are older males with no general symptoms except itching. Although the course is chronic, lesions improve slowly and heal only after years.     

挪威疥1例

患者女,60岁。全身弥漫性红斑脱屑1个半月,皮肤科情况:躯干及四肢见弥漫性水肿性红斑,其上泛发针尖至米粒大红色丘疹及丘疱疹,手背及指间潮红、肿胀、肥厚,上覆鳞屑,散在分布淡黄色厚痂,有特殊臭味。镜检见疥虫的虫体及虫卵。诊断:挪威疥;红皮病。     

Invasive dermatophytosis with lymph node involvement in an immunocompetent patient

A 23-year-old man presented with annular and arcuate, hyperpigmented, itchy, scaly plaques over the trunk and lower extremities for 5 years progressing to verrucous papules and nodules for the last 1.5 years. He also had nontender, inguinal and axillary lymphadenopathy. Skin and lymph node biopsies showed granulomatous inflammation and special stains demonstrated long septate hyphae. Tissue cultures grew Trichophyton verrucosum. The patient was treated with itraconazole 100 mg twice daily for 8 months, resulting in complete clearance of the lesions.