朗格汉斯细胞组织细胞增生症4例误诊分析并文献复习

目的分析朗格汉斯细胞组织细胞增生症(langerhans cell histiocytosis,LCH)临床特点及误诊原因,以降低误诊率,提高治疗效果。方法回顾分析在浙江大学医学院附属第二医院确诊的4例LCH的临床资料,分析误诊原因。结果 4例病例,3例成人,1例儿童,分别被误诊为"寻常型天疱疮、皮肤感染、慢性家族性良性天疱疮、尿布疹",后均经组织病理及免疫组织化学证实为LCH。结论朗格汉斯细胞组织细胞增生症学是一组可累及成人及儿童的少见病,临床表现复杂,皮损无特异性,容易误诊、漏诊,临床医生应加强少见病的学习认识,及时行组织病理及免疫组织化学检查,并全面评估有无系统受累。     

儿童朗格汉斯细胞组织细胞增生症2例

报道2007年在我科诊治的2例儿童朗格汉斯细胞组织细胞增生症,并结合文献对该病的临床表现、皮肤病理学特点、免疫组化特征及鉴别诊断进行总结分析.对怀疑儿童朗格汉斯细胞组织细胞增生症患者,应该尽早做皮肤病理及相关的免疫组化检查进行确诊.     

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...     

成人皮肤型朗格汉斯细胞组织细胞增生症

报告1例成人皮肤型朗格汉斯细胞组织细胞增生症。患者女,23岁。四肢、头部及面部大量丘疹、结节,无自觉症状2年。皮损组织病理检查示真皮全层片状、条索状单一核细胞浸润,免疫组化示肿瘤细胞CD1a(+)、Langerin(部分+)及Ki-67(约20%+)。透射电镜下肿瘤细胞内见不典型Birbeck颗粒。诊断:皮肤型朗格汉斯细胞组织细胞增生症。鉴于患者一般情况好,无其他器官累及,未予治疗,随访3个月,部分皮损变平及消退。     

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28例临床分析

探讨儿童朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床特点及治疗方法,以提高LCH的临床诊疗水平。方法:28例儿童LCH,确诊前均行组织病理检查,按Lavin-Osband分级和临床分型,I型和I级、Ⅱ级LCH患儿中单一骨损害采用手术刮除,单一皮肤损害、Ⅱ型、Ⅲ型、Ⅲ级、Ⅳ级采用泼尼松诱导化疗和随后长春新碱加泼尼松(VP)持续化疗。结果:LCH可累及多个系统和脏器,伴有不同的功能障碍,5例单一皮肤型LCH因皮疹减轻或消退家属拒绝化疗。7例单发的骨损害采用手术刮除后痊愈,10例单一皮肤型LCH和6例系统型LCH采用联合化疗。6周后总体疗效评价:治愈率46.43%;18个月后总体疗效评价:治愈率82.14%。结论:LCH是一组特异性以骨髓源性朗格汉斯细胞细胞(Langerhans cell,LC)和成熟的嗜酸性粒细胞增生为特征的先天性疾病,表现为骨、皮肤和淋巴结等局部器官或全身的LC的异常增生。LCH主要发生在儿童,成人少见,临床表现多种多样,经免疫组化可以获得正确诊断,但目前临床缺乏理想统一的治疗方案。泼尼松诱导化疗和随后长春新碱加泼尼松(VP)持续化疗治疗儿童LCH的疗效满意,不良反应、并发症少,值得临床推广。     

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。     

26例以皮疹为首发症状的儿童朗格汉斯细胞组织细胞增生症临床与病理分析

目的研究分析以皮疹为首发症状的儿童朗格汉斯细胞组织细胞增生症(LCH)的临床表现及组织病理特点,以提高LCH早期诊断率。方法对26例以皮疹为首发症状就诊的LCH患者的临床资料、组织病理、免疫组化结果进行综合分析。结果 26例患儿中,男16例(61.54%),女10例(38.46%),男女比例1.6∶1,2岁22例。临床上以湿疹、脂溢性皮炎、紫癜样皮疹多见。24例患儿合并其他系统受累,最常见为骨10例(38.46%),脾9例(34.62%),肺8例(30.77%)。免疫组织化学检查中,26例患儿CD1a、S-100均为阳性。结论 LCH发病越早,往往预后越差,皮疹可呈多种形态,提高皮肤活检率尽早完善各项系统检查,有助于儿童LCH的早期诊断,为其进一步的治疗提供依据。     

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.     

An 'eruptive' variant of juvenile xanthogranuloma associated with langerhans cell histiocytosis

The development of juvenile xanthogranuloma (JXG) as a sequel to langerhans cell histiocytosis (LCH) treated with chemotherapy is rare and the hypothesis is intriguing. This is a case of a 19-year-old woman who presented with progressive development of tan-red papules on the axilla and eyelids over a 1.5-year time span. A biopsy of an axillary lesion showed a prominent dermal infiltrate of foamy histiocytoid cells with occasional Touton-type multinucleate giant cells, consistent with JXG. Three years later, the patient presented with additional similar papules on the axilla and vulva as well as a painful mass in the pelvic bone and diabetes insipidus with an associated pituitary mass. An iliac crest bone biopsy showed an eosinophil-rich infiltrate admixed with histiocytoid cells with reniform nuclei, which expressed S100 and CD1a, consistent with a diagnosis of LCH. Nonetheless, an additional axillary papule was once again consistent with JXG, with negative reaction for S100 and CD1a with no Birbeck granules by electron microscopy. This case is unique by the co-existing presentation of multiple cutaneous JXG lesions and internally confined LCH lesions without an apparently associated chemotherapy, corroborating the concept that JXG and LCH may share a common histogenesis.     

Molluscum Contagiosum‐Like Presentation of Langerhans Cell Histiocytosis: A Case and Review

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation of Langerhans cells in the skin. A molluscum‐like presentation of cutaneous LCH is rare but important to consider for examination and management. We present an atypical molluscum‐like LCH case and review the literature for common features of this unusual presentation.     

Co‐existence of Langerhans cell histiocytosis and reticulohistiocytosis with initial presentation of skull lesions: A case report

Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder resulting from dysregulated clonal proliferation of Langerhans cells. Reticulohistiocytosis (RH) is another rare histiocytosis caused by the proliferation of histiocytes other than Langerhans cells. Co‐existence of LCH and RH in different organs and in the same skin area has not been reported. We present the case of a 20‐year‐old woman who initially had co‐existing bone LCH and cutaneous RH. After 1 year of chemotherapy with cytarabine, bone LCH significantly improved but cutaneous LCH developed in the same area where cutaneous RH was, resulting in hybrid LCH and RH of the skin. This unique history provides some evidence to support the theory that LCH and RH originate from the same stem cells and subsequently develop into hybrid LCH and RH of the skin in a cytokine environment influenced by chemotherapy. Repeat skin biopsies may be considered for adjusting treatment regimens in LCH patients whenever pre‐existing skin lesions progress.     

Extensive cutaneous Langerhans cell histiocytosis in an elderly woman

Langerhans cell histiocytosis (LCH), especially with involvement limited to the skin in adults is a rare entity. Primary cutaneous LCH in older patients usually follows a benign course and can regress spontaneously. We report a fatal case of disseminated cutaneous LCH in an elderly woman. A 64‐year‐old woman presented with confluenced erythematous macule and diffuse papuloscaling and papulocrusted lesions on the trunk that became erosive on the area of axillae and groins. Histopathological study of skin specimens showed extensive epidermotropic and folliculotropic, lichenoid infiltration of Langerhans' cells. Positive immunohistochemical staining for CD1a and S‐100 protein in Langerhans' cells, and numerous typical Birbeck granules in the cytoplasm of Langerhans' cells by electron microscopy study confirmed the diagnosis of LCH. Chest X‐rays and computed tomography scans showed mild interstitial pneumonia without a honeycomb appearance. The patient was diagnosed with LCH. Her general condition worsened rapidly and she died 1 month after diagnosis. Because extracutaneous involvement of LCH had been ruled out by laboratory and imaging investigations, we would like to believe this case should be classified as “malignant” LCH based on the clinical course.     

Adult‐onset systemic Langerhans cell histiocytosis mimicking inflammatory bowel disease: the value of skin biopsy and review of cases of Langerhans cell histiocytosis with cutaneous involvement seen at the Mayo Clinic

Background

Langerhans cell histiocytosis (LCH) is frequently known to involve multiple organ systems. However, gastrointestinal involvement by LCH is rare.

Methods

We describe a 68‐year‐old woman with a 3‐year history of intermittent diarrhea initially diagnosed as inflammatory bowel disease. She was subsequently found to have systemic LCH with involvement of the gastrointestinal tract, lungs, liver, and skin after skin biopsy was performed. A retrospective review of patients with cutaneous involvement of LCH seen at the Mayo Clinic over the past 15 years was conducted. The presence of systemic disease as well as specific organ system involvement was reviewed.

Results

Twenty‐four patients with cutaneous LCH were identified. Besides our case, one other patient with both gastrointestinal and cutaneous involvement was identified. This patient died at six months of age. No other adult‐onset cases were identified.

Conclusions

Gastrointestinal involvement with LCH is rare, can be easily misdiagnosed, and likely portends a poor prognosis. In patients with ill‐defined systemic symptoms, cutaneous exam and biopsy have the potential to diagnose systemic disease.     

Langerhans cell histiocytosis associated with breast carcinoma successfully treated with topical imiquimod

Langerhans cell histiocytosis (LCH) encompasses a group of disorders characterized by the proliferation and infiltration of Langerhans cells within internal organs and/or skin. There is often multiorgan involvement; isolated cutaneous LCH is less common. ¹ The aetiology of cutaneous LCH remains uncertain, and debate remains as to whether LCH represents a neoplastic condition or is simply reactive. We report a 53‐year‐old woman who developed isolated cutaneous LCH 15 months after being diagnosed with infiltrating ductal carcinoma of the left breast. The LCH was treated with topical imiquimod, resulting in clinical and histological resolution. Our case highlights the rare association between cutaneous LCH and breast carcinoma, and the clinical and histological response that can be achieved with topical imiquimod. After a diagnosis of LCH, patients require long‐term follow‐up, due to the risk of recurrence and/or development of a subsequent malignancy.     

Langerhans cell histiocytosis of skin: a clinicopathologic analysis of five cases

BACKGROUND AND AIMS: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. We retrospectively studied clinicopathologic features of this disorder in five cases. METHODS: Clinical and histopathological findings of five cases of cutaneous LCH were reviewed based on the hospital records. RESULTS: The age of patients ranged from 28 days to 5 years and M: F ratio was 1:1.5. Clinically, the diagnoses suggested were histiocytosis, varicella, transient neonatal pustular melanosis, keloid, sarcoidosis, seborrheic keratosis and LCH. The most common type of skin lesion was a generalized papular lesion. Histologically, all cases showed aggregates of large mononuclear histiocytes (Langerhans cells) with reniform, irregular, cleaved nuclei and abundant eosinophilic cytoplasm. There was multi-systemic involvement in two patients and single-system involvement in three patients. CONCLUSION: Cutaneous lesions may be the sole presenting feature of LCH. Diagnosis is based on demonstration of S-100 positive histiocytes.     

Langerhans cell histiocytosis associated with myelodysplastic syndrome in adults

BACKGROUND: Myelodysplastic syndrome (MDS) is a group of bone marrow disorders associated with dyplasia of myeloid elements that may have cutaneous manifestations including infections, vasculitis, Sweet's syndrome, pyoderma gangrenosum, erythema elevatum diutinum, and leukemia cutis. These cutaneous manifestations are attributed to the underlying bone marrow defect. Langerhans cell histiocytosis (LCH) is primarily a pediatric disease, and rarely LCH has been described in association with pediatric MDS. We are aware of only a single case report of LCH associated with MDS in an adult. METHODS: We report two new cases of LCH in elderly patients with underlying MDS. The specimens were examined by routine microscopy as well as immunohistochemical stains for S100 protein and CD1a. RESULTS: Both patients were elderly men with established diagnoses of MDS. One presented with a solitary pruritic papule while the other had a 2-year history of erythematous papules involving the trunk and extremities. Histologic examination revealed intraepidermal and dermal collections of mononuclear cells with reniform nuclei. The cells were strongly positive for S100 and CD1a, confirming their identity as Langerhans cells. CONCLUSION: Cutaneous LCH may be associated with underlying MDS in adults and should be considered in the differential diagnosis of cutaneous eruptions in patients with MDS.     

Cutaneous Langerhans cell histiocytosis with subsequent development of haematological malignancies. Report of two cases

Adult cutaneous Langerhans cell histiocytosis (LCH) is a rare disease. We report two cases illustrating the variability of the clinical presentation and the response to treatment. In both cases a remission was achieved: in one case a partial remission with psoralen plus UVA irradiation (PUVA) and methotrexate plus topical corticosteroid ointment; in the other case by treatment with thalidomide. Despite a therapeutic response, both patients later developed haematological malignancies: a chronic myelo-monocytic leukaemia and an acute lymphatic leukaemia. In conclusion, patients with adult cutaneous LCH should be monitored carefully so that a secondary malignancy is not overlooked.     

Adult Langerhans cell histiocytosis limited to the skin

BACKGROUND: The incidence of Langerhans cell histiocytosis (LCH) is 4-5 per million in children with only 30% of this number having an adult onset. While dermatological manifestations occur in as many as 50% of cases, disease limited to the skin is uncommon among reported cases of adult LCH. OBJECTIVES AND METHODS: To present 3 new cases of adult LCH and a review of the literature of isolated cutaneous LCH in adults. RESULTS: Three adults with scalp, vulvar and generalized LCH lesions had refractory responses to treatment. CONCLUSIONS: LCH may present with unusual cutaneous manifestations limited to the skin in adults. Optimal treatment has not yet been determined.