先天性自愈性朗格汉斯组织细胞增生症

报告1例先天性自愈性朗格汉斯组织细胞增生症.患儿男,2个月.出生时即有全身多发性皮肤结节,皮肤科检查:左耳后、左面部、胸部、背部、左下肢、右示指共6处鲜红色结节,中央形成火山口状溃疡,组织病理示结节由梭形细胞构成,部分细胞胞质丰富,嗜酸性,可见多角、多核瘤巨细胞,间质纤维丰富,局部瘤细胞增生活跃.免疫组化:CD1a(+...     

传染性软疣样先天性自愈性朗格汉斯组织细胞增生症一例

报道一例传染性软疣样表现的先天性自愈性朗格汉斯组织细胞增生症。患儿,女,5个月。出生4个月后周身出现多发肤色扁平小丘疹,表面光滑,边界清楚,部分中央脐凹样改变。组织病理示真皮乳头内单一核细胞浸润,免疫组化：CD1a(+),S100(+),Langerin(+)。病理取材1周后所有皮疹均消退,结合患儿临床症状及随访结果诊断为先天性自愈性朗格汉斯组织细胞增生症。     

儿童朗格汉斯细胞组织细胞增生症1例

1 病历摘要 患儿男,4个月.因头颈部、躯干丘疹3个月,发热4 d就诊.患儿3个月前,无明显原因头颈出现红色丘疹,无瘙痒,皮损渐累及躯干,以腋窝、腹股沟明显.多次在外院诊断为"湿疹"、"念珠菌病",予以对症处理后,皮损部分消退,但反复出现.     

女童外阴原发性朗格汉斯组织细胞增多症一例

患儿女,5岁3个月,因发现外阴皮疹5个月于2009年4月15日入院.入院前5个月,患儿家长无意中发现其外阴可见数个米粒大小红疙瘩,无痛痒等不适,亦无发热、尿频、尿急等症状,曾经自以为湿疹,外用曲安奈德益康唑乳膏,治疗无效.2周前在我科门诊行外阴皮疹组织病理检查,结果:局部真皮浅层片状增生的组织细胞,并向上侵犯表皮(图1),可见核沟,部分核呈肾形(图2),其间见少量淋巴细胞、嗜酸粒细胞.     

成人朗格汉斯细胞组织细胞增生症1例

患者男,48岁,肛周皮肤增生性斑块半年。皮肤科情况:肛周见裙边样增生性斑块,边缘游离,表面光滑。皮损组织病理示:表皮基本正常,真皮乳头水肿,可见大量浆细胞浸润,中下层可见大量增生单个核细胞成结节状浸润,胞质丰富淡染,核椭圆形或肾形,部分可见核沟,并可见大量的嗜酸性粒细胞及淋巴细胞浸润。免疫组织化学示:CD1a(+)、S-100(+)、CD68(+),Ki-67(40%+)。诊断:朗格汉斯细胞组织细胞增生症。治疗:给予口服沙利度胺50 mg,2次/d治疗,3个月后肛周皮损明显消退。     

发疹性朗格汉斯细胞组织细胞增生症1例

报告1例发疹性朗格汉斯细胞组织细胞增生症。患者男,27岁。四肢、躯干皮肤粟粒至黄豆大播散性丘疹、结节4年余,不伴系统损害。进展缓慢。皮损组织病理检查示真皮团块状组织细胞浸润,免疫组化染色结果示S-100蛋白（＋）,CD1a（＋）,LYSO（＋）,电镜下组织细胞内找到Birbeck颗粒。经糖皮质激素软膏外用,皮损有所消退,但停药后复发。随访2年,患者皮损进展缓慢,其他系统未累及。     

传染性软疣样表现的先天自愈性朗格汉斯细胞组织细胞增生症5例皮肤镜特点及临床与组织病理分析

目的:总结5例传染性软疣样表现的先天自愈性朗格汉斯细胞组织细胞增生症临床特征、皮肤镜下表现及组织病理学特点.方法 :采用回顾性分析对昆明医科大学附属昆明市儿童医院皮肤科5例传染性软疣样表现的先天自愈性朗格汉斯细胞组织细胞增生症患儿的临床资料、皮肤镜图片、组织病理及免疫组化染色资料进行分析总结.结果 :5例患儿中,男3例...     

朗格汉斯细胞性组织细胞增生症研究进展

朗格汉斯细胞性组织细胞增生症是一种少见的疾病。近来研究发现吸烟、病毒感染及遗传因素与部分患者发病可能密切相关,T细胞在朗格汉斯细胞性组织细胞增生症的发生中起重要作用。该疾病不仅累及皮肤粘膜,而且可累及中枢神经系统、消化系统、肺脏及甲状腺等多个系统器官。单个系统受累时预后较好,多系统受累时则预后不良。对于朗格汉斯细胞性组织细胞增生症的治疗,应根据不同的病情采取适宜的措施。     

朗格汉斯组织细胞增生症1例并文献复习

报告1例以皮肤损害就诊的朗格汉斯组织细胞增生症.患儿男,2岁半,因头面、躯干、下肢红斑、丘疹、脓疱一年半入院.皮肤组织病理检查:大量单个核细胞弥漫分布于真皮乳头层并向表皮侵入,单个核细胞体积较大,胞质淡染,核折叠成肾形,中央空泡化,可见线状沟,呈咖啡豆样外观.免疫组织化学法:CD1α及 S-100染色阳性.诊断为朗格汉斯组织细胞增生症.同时结合相关文献对朗格汉斯组织细胞增生症的临床表现、诊断和预后等进行分析.     

成人皮肤朗格汉斯细胞组织细胞增生症1例

目的探讨成人皮肤朗格汉斯细胞组织细胞增生症(LCH)的临床、组织病理学、免疫表型及电镜特征。方法报告1例罕见的成人皮肤朗格汉斯细胞组织细胞增生症,并结合文献分析其临床病理特征。结果该例先后经过4次病理活检,组织学特点为真皮全层弥漫分布的朗格汉斯细胞。瘤细胞表达CD1a、S-100、部分表达Langerin。电镜检查发现Birbeck颗粒。结论成人皮肤弥漫性LCH罕见,误诊率高。确诊应结合组织学特征、免疫表型及超微结构检查综合分析,疾病的预后与发病年龄、受累器官的多少有关。     

Late-onset self-healing reticulohistiocytosis: pediatric case of Hashimoto-Pritzker type Langerhans cell histiocytosis

An 8-year-old otherwise healthy girl presented with a 3-month history of multiple asymptomatic, reddish-brown papules over the face and upper limbs. Histopathological and immunohistochemical examinations demonstrated an infiltrate of mononuclear cells containing abundant histiocytic cells in the dermis, and microabscess-like accumulation of the histiocytic cells in the epidermis. The histiocytic cells were positive for antibodies against S-100 protein and CD1a, but negative for anti-CD68. Lag and anti-langerin monoclonal antibodies reacted more weakly with these histiocytic cells than with Langerhans cells in the surrounding epidermis. The skin lesions spontaneously regressed within the following 3 months, and neither systemic involvement nor local recurrence was observed during the next 10 months. This case should be categorized as congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker), although the onset was not early in life.     

朗格汉斯细胞组织细胞增生症

报告1例朗格汉斯细胞组织细胞增生症.患儿男,10个月.躯干部出现丘疹2个月,经组织病理及免疫组化检查证实为朗格汉斯细胞组织细胞增生症.     

朗格汉斯细胞组织细胞增生症1例

患儿6个月,生后2个月开始腹部,头皮,腹股沟部出现浅红色丘疹,头皮有脂溢性结痂。腹股沟、会阴区出现糜烂,局部发生溃疡。经病理及免疫组化检查证实为朗格汉斯细胞组织细胞增生症。     

老人皮肤朗格汉斯细胞组织细胞增生症1例

朗格汉斯细胞组织细胞增多症（LCH）是以朗格汉斯细胞增生为特征的多系统侵犯的反应性增生性疾病,小儿多见,成人少见,老人罕见,对1例62岁老人的红皮病样损害伴明显瘙痒患者行临床,皮肤和淋巴结病理及免疫组化等检查,诊断LCH,该患者经合理治疗,取得满意疗效。     

黄色肉芽肿样单发型朗格汉斯细胞组织细胞增生症一例

朗格汉斯细胞组织细胞增生症（Langerhans cell histiocytosis, LCH）是一种少见的病因不明的疾病，表现为单发型皮损的朗格汉斯细胞组织细胞增生症更是罕见,本文报道一例。该患儿7个月，表现为右侧肩部单发黄红色结节2个月，皮损与黄色肉芽肿非常相似，极易误诊为黄色肉芽肿。     

Unilesional self‐limited Langerhans cell histiocytosis: a case report and review of the literature

Self‐limited Langerhans cell histiocytosis (LCH) represents a rare, congenital, cutaneous form of LCH associated with a good prognosis. Only 35 cases of solitary lesion self‐limited LCH have been reported. Herein, we present an additional case in a 3‐month‐old boy who presented with an isolated pigmented nodule on his left posterior shoulder, which had been present since birth. Punch biopsy showed histopathologic features of LCH with positive CD1a and CD68 stains. Further examination and investigation showed no features of systemic involvement. The lesion spontaneously resolved within 2 months, and there has been no evidence of recurrence on follow up. As several cases of recurrence and complications have been reported in self‐limited LCH, we emphasize the need for long‐term, and perhaps indefinite, follow up for symptoms and signs associated with LCH in these patients.     

Langerhans cell sarcoma: a case report

Primary cutaneous neoplasms of histiocytes and dendritic cells are rare. Langerhans cells are a subset of antigen‐presenting dendritic cells. Neoplasms of Langerhans cells are classified into cytologically benign Langerhans cell histiocytosis and cytologically malignant Langerhans cell sarcoma. Langerhans cell sarcoma is a rare entity characterized by multiorgan involvement and an aggressive clinical course. To date, only 30 cases of Langerhans cell sarcoma, including the present case, have been reported. We report a new case of Langerhans cell sarcoma that presented with multifocal cutaneous involvement. Diagnosis was done based on histopathological, immunohistochemical evaluation, as well as ultrastructural analysis identifying the presence of Birbeck granules. Our case represents a new case of this extremely rare, overtly aggressive neoplasm of Langerhans cells. Within 2 years of diagnosis, the patient developed metastatic disease and consequently died. Early recognition is important because of the tendency of Langerhans cell sarcoma to recur and metastasize. Therefore, ancillary techniques such as immunohistochemical and ultrastructural studies to confirm the diagnosis are very advantageous.     

Congenital Langerhans cell histiocytosis presenting in a 27‐week‐gestation neonate

Langerhans cell histiocytosis is exceedingly rare in premature infants, and the few cases reported suggest a poor prognosis with systemic involvement. We present a case of Langerhans cell histiocytosis limited to a single cutaneous lesion, presenting in a 27‐week‐gestation infant, which is the youngest gestational age of reported Langerhans cell histiocytosis cases. The lesion showed spontaneous resolution by 41 weeks corrected gestational age, and systemic involvement was absent, demonstrating a mild course of skin‐only Langerhans cell histiocytosis in a premature infant.