皮损形态类似于光泽苔藓的朗格汉斯细胞组织细胞增生症一例

报道1例表现为光泽苔藓样皮损的朗格汉斯细胞组织细胞增生症。患儿女,19个月。因前胸、后背多发针尖大小丘疹伴多饮、多尿1个月余就诊。皮损临床表现为全身泛发性光泽苔藓样丘疹。垂体平扫+增强MRI提示：垂体后叶短T1信号消失。皮损组织病理显示表皮角化过度,真皮浅层密集组织细胞浸润,细胞核呈肾形,胞浆丰富。免疫组化：CD1a、S-100、CD68均阳性。结合临床、组织病理和免疫组化检查,诊断为朗格汉斯细胞组织细胞增生症。全身化疗后,皮损完全消退,目前仍在随访中。     

Letterer-Siwe一例

朗格汉斯细胞组织细胞增生症(LCH)又称为组织细胞增生症X、朗格汉斯细胞病和朗格汉斯细胞肉芽肿.可分为急性泛发性LCH(即Letterer-Siwe病,莱特勒-西韦病)、多灶性慢性LCH (即Hand-Sch黮lerchristian病,简称H-S-C病)和局灶性LcH(即嗜酸性肉芽肿).现将我院收治1例5个月大的莱特勒-西韦病患儿报告如下.     

Letterer-Siwe病1例

朗格汉斯细胞组织细胞增生症是一组罕见的原因未明的疾病,又称为组织细胞增生症X、朗格汉斯细胞病和朗格汉斯细胞肉芽肿,其中Letterer-Siwe病极为少见,我科收治1例,现报告如下.     

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治疗进展

朗格汉斯细胞组织细胞增生症是朗格汉斯细胞克隆性增生形成的一组有多种临床表现的病谱性疾病,该病可累及单器官或多器官,如骨骼、皮肤、肺等,成人发病率较低。本文主要综述了成人皮肤朗格汉斯细胞组织细胞增生症的治疗进展,希望能为临床诊治工作提供参考。     

朗格汉斯细胞组织细胞增生症并发多系统受累

报道1例多系统受累的朗格汉斯细胞组织细胞增生症。患者男,20岁,甲变形、部分缺如8年余,皮损及甲状腺增大5年,肝功能异常1年,黄疸5个月,多饮多尿1个月。皮肤科检查:面、颈、躯干及四肢充血性、出血性浸润性暗红色丘疹、斑块,甲变形缺如。面部皮损组织病理检查:真皮内见大片浸润性分布的肿瘤细胞,细胞胞质丰富,红染,核大,呈不规则形,核分裂象可见;免疫组织化学染色:S-100蛋白,CD1a均阳性。诊断:朗格汉斯细胞组织细胞增生症。治疗:予化疗及对症治疗,疗效不佳。     

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生症是一组以朗格汉斯细胞异常增生为主要病理特征的疚病,同时累及甲和外阴部位者临床罕见.     

全身泛发型幼年性黄色肉芽肿一例

幼年性黄色肉芽肿（JXG）是儿童中常见的一种非朗格汉斯细胞组织细胞增生症。一般表现为皮肤丘疹或结节,累及全身者罕见,仅发生于3.9%~5%的患者~（[1,2]）。其病因不明。本文报道1例四肢、躯干、眼眶等全身泛发型黄色肉芽肿。     

儿童朗格汉斯细胞组织细胞增生症42例临床分析

朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是指一组病因未明的以朗格汉斯细胞(Langerhans cell,LC)异常增生为主要病理特征的疾病.     

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。     

Acquired mucosal indeterminate cell histiocytoma

Indeterminate cell histiocytosis is an exceptional and controversial entity with variable clinical, histopathologic or immunohistochemical findings, sharing morphologic and immunophenotypic features from both Langerhans and non-Langerhans cell histiocytoses. Neoplastic cells express S-100 and CD1a antigens, but lack Birbeck granules. It has been reported in both adults and children, as solitary or multiple cutaneous lesions with rare extracutaneous involvement. We describe a 12-year-old boy with an indeterminate cell histiocytosis manifesting as a solitary verrucous papule on the mucosa of the glans penis. The morphologic features and diagnostic criteria of cutaneous indeterminate cell histiocytic proliferations are reviewed. The possible relationship between indeterminate cell and Langerhans cell histiocytoses is discussed.     

Solitary congenital self-healing reticulohistiocytosis in monozygotic twins

We report monozygotic twins with congenital self-healing reticulohistiocytosis, whose lesions initially presented as hemorrhagic bullae at birth with rapid progression into crusted papules the following day. Physical examination disclosed crusted papules on the right side of the neck of twin 1 and a similar solitary lesion on the lateral side of the right thumb of twin 2. Excisional biopsy specimen findings of the neck and thumb lesions were consistent with Langerhans cell histiocytosis, which was further confirmed by positive CD1a staining. The lesions resolved completely by 2 months with no evidence of recurrence or systemic involvement. Congenital self-healing reticulohistiocytosis is a rare, self-limited form of Langerhans cell histiocytosis. Although familial clustering in Langerhans cell histiocytosis was previously reported, to the best of our knowledge there is no report suggesting familial clustering in congenital self-healing reticulohistiocytosis. Our patients are interesting in terms of raising the question of whether the presence of congenital self-healing reticulohistiocytosis in monozygotic twins is implicative of a genetic role in its pathogenesis.     

Cutaneous Langerhans cell histiocytosis presenting with hypopigmented lesions: Report of two cases and review of literature

Langerhans cell histiocytosis is a rare group of disorders that results from the abnormal proliferation and accumulation of dendritic‐derived cells in various organs of the body, such as the skin and bones. Hypopigmented macules are a rare cutaneous presentation of Langerhans cell histiocytosis that may pose a diagnostic dilemma when no other findings of Langerhans cell histiocytosis are present at the time of examination. We present 2 cases of the hypopigmented variant of Langerhans cell histiocytosis, including a case with histopathologic features of regression, and a review of the literature. These cases highlight the importance of including Langerhans cell histiocytosis in the differential diagnosis of an infant with hypopigmented macules and papules.     

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.     

Solitary Congenital Erosion in a Newborn: Report of a Solitary Congenital Self-Healing Reticulohistiocytosis

Congenital self-healing reticulohistiocytosis (CSHRH) is a rare, cutaneous, self-limited form of Langerhans cell histiocytosis. Whereas multiple lesions are common, a solitary lesion is rare. A 14-day-old neonate presented with a solitary, 5-mm, oval, reddish, and eroded papule with crust on the left thigh that had existed since birth. No systemic involvement was found. Histopathology revealed dense infiltration of large histiocytes with scattered eosinophils and lymphocytes in the dermis. Immunohistochemistry showed S-100 and CD1a positivity. Two months later, the skin lesion involuted spontaneously, without evidence of recurrence and extracutaneous involvement. On the basis of the characteristic clinical course and typical histopathological findings, a diagnosis of solitary CSHRH was made.     

Isolated Langerhans Cell Histiocytosis of the Vulva in an Infant

Abstract: Langerhans cell histiocytosis affecting only the vulva of a child is very rare. We report a 1‐year‐old female infant with isolated Langerhans cell histiocytosis presenting as pruritic papules confined to the vulva, confirmed by histopathology with immunohistochemical staining.     

Solitary nodular Langerhans cell histiocytosis

We report a 3-month-old girl with a solitary variant of congenital self-healing reticulohistiocytosis, one type of Langerhans cell histiocytosis.     

Langerhans cell histiocytosis of the mandible in a six-year-old child

Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported. We report a six and a half-year-old patient with Langerhans cell histiocytosis--solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and had a fatal outcome.     

Solitary congenital self-healing reticulohistiocytosis

Congenital self-healing reticulohistiocytosis (CSHR) was first described in 1973 by Hashimoto and Pritzker. Since then, both multiple and solitary forms have been described. We report a further case of solitary congenital self-healing reticulohistiocytosis. CSHR is a rare disorder which frequently presents a diagnostic dilemma in the newborn. Nodular forms of systemic Langerhans cell histiocytosis may present in a manner similar to CSHR. Because their differentiation on histopathological grounds is impossible, physical examination, laboratory investigations, and follow-up are necessary to ensure a lack of systemic involvement. Although previously thought to represent a rare variant of CSHR, solitary lesions of CSHR account for almost 25% of reported cases. A review of the literature relating to solitary CSHR, and a consideration of the differential diagnosis of a solitary congenital nodule, are also presented.     

Is Langerhan Cell Histiocytosis Complicated with Hydrops Fetalis Exclusively Lethal in Premature Neonates?

Langerhans cell histiocytosis is a rare disease and is lethal in premature neonates. A male premature neonate born at gestational 33 weeks presented with generalized vesicles, hydrops fetalis with pleural effusion, bilateral cataracts, and severe respiratory distress syndrome complicated with persistent pulmonary hypertension. Skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis.