急性发病的原发性皮肤毛霉病1例

患者女,71岁。左小腿血疱、溃疡、坏死伴疼痛20余天。皮损组织病理示:表皮及真皮全层坏死,真皮感染性肉芽肿改变,其中见粗大无分隔的菌丝。经真菌培养和分子生物学鉴定为不规则毛霉。经伊曲康唑胶囊抗真菌治疗后好转。     

Klippel-Trenaunay-Weber综合征1例

患者男,22岁.因右上肢紫红色斑22年于2012年2月2日来我院就诊.患者于出生时右上肢即有散在、边界稍清楚、形状不规则的淡紫红色斑,且整个右上肢较左上肢粗大,随着患者生长发育,其皮损逐渐融合并累及整个右上肢,右侧胸部、肩部、肩胛部,左胸部及小部分左上臂前侧,形成大片边界清楚不规则形颜色较均匀紫红色斑,且患者自觉右上肢胀痛及沉重感,其肌力大于左上肢,汗液较左上肢多.     

多发性疣状角化不良瘤一例

46岁男性患者。额部、左颞部角化性丘疹1年,缓慢增大。皮损组织病理示:表皮角化过度伴角化不全,不规则增生,棘层松解,基底层上方形成裂隙,可见谷粒及圆体。诊断:疣状角化不良瘤。肿物切除术后愈合良好,无复发。     

炎性线状疣状表皮痣3例

例1男,36岁,右足小趾、足背外缘沿小腿屈侧至腘窝出现暗红色角化性丘疹、斑块伴瘙痒32年.例2女,4岁,左下腹及腹股沟红色或红褐色丘疹、斑丘疹沿Blaschko线分布至外阴部,伴瘙痒3年半.例3男,26岁,右手背、手指、上肢至肩部暗红色丘疹、斑丘疹伴瘙痒3年.该3例皮损组织病理均示:表皮角化过度伴角化不全,棘层肥厚,呈...     

红皮病型银屑病并发鳞状细胞癌1例

患者男,57岁.周身反复鳞屑性红斑丘疹伴瘙痒30年,左下肢外伤后溃疡半年.溃疡处组织病理示:表皮增生,灶性角化不全,真皮全层可见大量鳞状细胞团,角珠易见,伴有较多炎性细胞浸润.诊断:红皮病型银屑病;鳞状细胞癌(SCC).     

Multiple papillary hidradenoma: a case report

报告1例发生于左上肢的多发性乳头状汗腺瘤.患者女,45岁.左上肢线状排列的结节伴瘙痒7年.皮肤科检查见左上肢数个直径2～3 cm淡红色结节,呈线状排列,质地坚实,部分皮损表面破溃、结黄褐色痂.组织病理检查示真皮浅层及中层内肿瘤由大小不一的腺管、腺泡、复杂折叠的乳头状结构构成,腺腔和乳头状结构内衬单层柱状上皮,外围见一层小立方形肌上皮细胞,符合乳头状汗腺瘤.     

表现为红斑结节及多毛的丛状血管瘤

报告1例以红斑结节伴多毛为主要表现的丛状血管瘤.患儿女,8岁.左上肢结节伴多毛和疼痛2年.皮肤科检查可见左前臂数个直径0.5～2 cm的暗褐色结节,部分结节萎缩.组织病理检查示真皮内散在毛细血管组成的团块,呈丛状或卵圆形,可见圆形或不规则形的管腔,含有较多的内皮细胞和周皮细胞,细胞无异形性.免疫组化染色结果示内皮细胞CD34、CD31阳性,周皮细胞平滑肌肌动蛋白(SMA)表达阳性.     

疣状表皮发育不良伴多发Bowen病1例

患者女,56岁。全身褐色扁平疣状丘疹40年,面颈部多发疣状增生物8年。腹壁、左前臂及手背皮疹组织病理示:角化过度,棘层增厚,表皮上部可见弥漫性细胞空泡化,细胞大小不一,胞质呈钢灰色。额面部皮疹组织病理示:表皮角化过度伴角化不全,棘层肥厚,肿瘤细胞位于表皮内,全层细胞排列紊乱,部分不典型;毛囊和汗腺导管细胞核大深染,可见异型改变。诊断为疣状表皮发育不良伴多发Bowen病。     

累及中枢神经系统的皮肤CD30~+间变性大细胞淋巴瘤1例

患者男,75岁,反复皮肤肿块11年。1个月前第3次复发伴语言、记忆和生活自理能力减退。头颅MRI示:左额叶,右顶叶多发占位,颅内淋巴瘤浸润。左大腿肿块组织病理示:真皮内大量异型细胞,体积较大,形态不规则,核扭曲,核分裂相可见;免疫组化:CD3(+),UCHL-1(+),CD30(+),ALK(-),MIB-1>90%。诊断:皮肤CD30+间变性大细胞淋巴瘤。中枢神经系统(central nerve system,CNS)累及可以是皮肤间变性大细胞淋巴瘤发生皮外累及的唯一部位,患者的意识改变是进行CNS筛查的重要提示。     

Ⅰ型神经纤维瘤病

报告1例以丛状型神经纤维瘤为典型临床表现的Ⅰ型神经纤维瘤病.患儿女,5岁.左上肢巨大褐色斑片伴毛发密集生长5年.皮肤科检查:颈胸部左侧到上肢巨大褐色斑片,其上密集增粗增多的毛发,全身散在多发的咖啡斑,下背部皮下结节以及双上肢粗细不等.左肱骨X线检查示左侧肱骨弯曲,鹰嘴窝扩大,局部骨质缺损.CT示左肱骨形态欠规则,鹰嘴窝扩大,左侧尺桡骨上段局限性骨质缺损伴软组织密度影填充.皮损组织病理:真皮层可见界限清楚但无包膜的肿瘤,由大量梭形瘤细胞组成,核细长,波浪状嵌在基质中.结合国内外文献对Ⅰ型神经纤维瘤病的病因、临床表现、并发症及治疗进行讨论,从而加强对该病的认识.     

河南不规则毛霉致原发性皮肤毛霉病一例

【摘要】 患者女,50岁。右上肢皮肤结节、斑块、红肿、溃烂6年,右臀部斑块2年,右面颊斑块1年。曾单独口服特比萘芬、伊曲康唑治疗无效。右上肢及臀部皮损组织病理及PAS染色均可见真皮内宽大呈直角分支菌丝。真菌培养及分子生物学鉴定为不规则毛霉。联合伊曲康唑和特比萘芬治疗2个月仍无效,改为静脉滴注两性霉素B（总量1 060 mg）治疗,患者右面颊、右臀部斑块肿胀逐渐消退,右上肢包括右手背斑块、红肿、坏死、黑痂消退,皮损愈合成瘢痕,取右上肢皮肤组织再次行真菌培养阴性。随访1年未复发。     

不规则毛霉感染引起的皮肤毛霉病20例回顾性分析

目的通过对不规则毛霉感染病例的回顾性分析,探讨不规则毛霉感染引起皮肤毛霉病的流行病学、易感因素、临床表现、诊断方法及治疗策略,提高临床医生的警惕性及诊治经验。方法对检索到的20例不规则毛霉感染病例资料进行总结、分析。结果 20例不规则毛霉感染患者均表现为皮肤毛霉病,仅1例同时伴有肺部感染。15例来自中国,1例有糖尿病史,1例有白血病史,10例有外伤、手术或叮咬史。皮损几乎均出现在暴露部位,其中鼻面部13例,四肢7例,病程多呈慢性。早期表现为红斑、丘疹、结节,后期可发展成溃疡、坏死、黑痂及骨质破坏。经真菌镜检、培养、组织病理、ITS区测序等检查证实为不规则毛霉。两性霉素B及其脂质体单独或联合伊曲康唑治疗预后好。结论不规则毛霉感染多发生于免疫正常者,外伤、烧伤、手术及叮咬是最主要诱发因素。感染主要累及鼻面部及四肢皮肤暴露部位,以红斑、坏死为主要表现,极少累及内脏。真菌培养及组织病理检查可以诊断该病,ITS区测序可明确鉴定到种。及早诊断并给予清创及两性霉素B是治疗成功的关键。     

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。     

肝移植术后根霉病1例

报告1例皮肤根霉病。患者男，50岁。因丙型肝炎、肝硬化接受原位肝移植术。术后第12天，右上肢前臂出现红斑、肿胀并渐扩大，皮肤呈黑色坏疽样外观。右上肢、肩部、腋下、胸背疼痛。皮损组织病理检查示血管周围有粗大、无分隔直角菌丝，血管内真菌栓塞。真菌学检查证实为接合菌病根霉属。后经聚合酶链反应(PCR)鉴定为小孢根霉。对上肢感染组织行清创术、截肢，并予以两性霉素B脂质体治疗3周。经治疗肿胀减轻，疼痛消失，目前仍在治疗中。     

点状瘢痕型疣状表皮发育不良1例

报告1例以少见的点状瘢痕为表现的疣状表皮发育不良。患者男,17岁。面颈、躯干、上肢大量点状轻微凹陷的斑疹12年。皮损组织病理示:表皮中上部有明显的弥漫性细胞空泡化。诊断:疣状表皮发育不良(点状瘢痕型)。予重组人α-2b干扰素凝胶外搽治疗。     

持久性色素异常性红斑一例

患者,男,53岁。颈部、双上肢青灰色斑疹进行性加重5年。系统检查未见明显异常。组织病理示：表皮大致正常,基底层细胞局灶性空泡化,真皮浅层少量炎细胞浸润,可见较多噬黑素细胞。诊断：持久性色素异常性红斑。     

Cutaneous and visceral sarcoidosis. Apropos of an exceptional form

We present here an exceptional case of cutaneous and vascular sarcoidosis associated with lesions of the liver, spleen and lymph nodes. The disease began when this male patient was 27 years' old and gradually extended over 35 years, despite long-term systemic corticosteroid therapy. Clinically, the initial cutaneous lesions were atrophic, erythematous and squamous, resembling those of erythroderma; they were located on the skin of the right popliteal fossa, the left arm, the neck, the upper part of the chest and around the waist. Subsequently, they spread slowly to involve almost the entire skin, except for the face, right upper limb, left lower limb, hands and feet. The most ancient of these lesions were distinctly black. The skin was paper thin due to complete disappearance of the subcutaneous tissue. Left temporo-parietal alopecia of the cicatricial type developed towards the end of the patient's life. The nails remained normal throughout, and there were no other cutaneous signs of sarcoidosis. Initially, the venous network was clearly visible beneath the atrophic skin, but later on, the veins became grossly dilated and sacculated in those areas which had first been invaded by skin atrophy. Phlebography of the left upper limb confirmed the venous dilatation, while arteriography of the upper limbs showed multiple sacciform aneurysms of the subclavian, axillary and brachial arteries, with distal thrombosis of the latter vessel. Histology showed typical lesions of sarcoidosis in a vein; no arterial biopsy was performed. The muscles had a nodular and sclerous appearance; amyotrophy developed in the last stages of the disease; tendons and joints were normal.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cutis marmorata telangiectatica congenita: crossed limb dimelia]

We report a case of localized cutis marmorata telangiectatica congenita involving the right upper limb and the left lower limb. This is a rare distribution, with only one case in the literature. The patient, a 22-month old boy, showed no other abnormality.     

Becker nevus on the lower limb: case report and review of the literature

BACKGROUND: Becker nevus is a hamartoma with brown hyperpigmentation and hypertrichosis that usually affects young males. It has a predilection for the upper half of the trunk and proximal upper extremity. OBJECTIVE: This is a case report of a 38-year-old male with a Becker nevus without hypertrichosis on the lower limb and a review of the literature. RESULTS: Including our case report, 12 cases of Becker nevus on the lower limb were identified in the English literature (8 males and 4 females). It has never been reported below the knee. In males, the lesion was not associated with other anomalies, and all but two showed hypertrichosis (six of eight). In 50% (two of four) of women, it was associated with localized lipoatrophy. In one woman, it presented without hypertrichosis. CONCLUSION: The presence of Becker nevi on the lower limb is unusual, and hypertrichosis is not always a feature. Larger studies are required to look into the true prevalence of this presentation and its prognostic value as a marker for other anomalies.     

海分枝杆菌感染一例

患者,女,57岁。右上肢结节3个月。组织病理检查为感染性肉芽肿性改变。PAS未见菌丝和孢子结构,抗酸染色阴性。海分枝杆菌qPCR阳性。诊断：海分枝杆菌感染。给予多西环素、克拉霉素、利福平等治疗,皮损逐渐消退。