Primary Cutaneous Langerhans Cell Histiocytosis Treated with Photochemotherapy

A 23-year-old man with Langerhans cell histiocytosis presented with asymptomatic, purplish, slightly scaly, confluent papules of one year's duration. Histological studies of biopsy specimens revealed a dense infiltrate of histiocytic mononuclear cells beneath the epidermis; these cells reacted strongly with anti-S-100 antibodies. Extensive investigations failed to detect systemic involvement. He was treated with repeated oral 8-methoxypsoralen (8-MOP) plus ultraviolet A (PUVA) therapy three times a week for two months and then once or twice with maintenance phototherapy. The lesions did not recur during the four-month follow-up period.     

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.     

Self-Healing Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption

apd: 23 November 2001     

Langerhans Cell Histiocytosis (Histiocytosis X) with Morphologic Expression of Desmosomes and Microvillous Structures

An unusual case of Langerhans cell histiocytosis in a 7-year-old female is presented. She had ultrastructural evidence of desmosomal biogenesis and formation of gland-like structures by lesional cells; their apical plasma membranes were folded into large numbers of microvilli. Despite the presence of these structures characteristic of epithelial cells, an infiltrated plaque on the abdominal skin of this patient was interpreted as cutaneous involvement of multiple system Langerhans cell histiocytosis because the immunohistochemical staining of the lesional cells for CD1a, S100, PNA, CD4, EN-4, and HLA-DR was positive, and numerous Birbeck granules were ultrastructurally identified in some lesional cells. Other clinical data included the presence of scaly erythematous skin lesions on the forehead and lytic osseous lesions in the maxilla, which were also histologically diagnosed as Langerhans cell histiocytosis. The absence of any internal malignancy in this patient readily ruled out the other diagnostic possibility of a metastatic adenocarcinoma showing glandular differentiation with brush border morphogenesis. The possibility that the desmosome-linked lesional epithelioid cells were actually cells of sweat glands entrapped in the histiocytic proliferation was also ruled out. The functional significance of the desmosomes and microvillous structures in the present case of Langerhans cell histiocytosis remains to be clarified. Awareness of this variant of Langerhans cell histiocytosis will be important for averting potential misdiagnosis in favor of epithelial tumors, especially metastatic adenocarcinomas.     

Langerhans Cell Histiocytosis: A Case Report and Glimpses into Its Nomenclature

Class I Langerhans cell histiocytosis (LCH) is described in a two- and a half-year-old male. The initial expression of the disease was conspicuous by the presence of extensive pustular crusted rash and, later in its course, by purpura, diabetes insipidus, otitis media, enlargement of the liver and spleen, and infiltration into the lungs. The patient expired due to extensive hemorrhage over 24 hours.     

表现为结节性损害的老年皮肤朗格汉斯细胞组织细胞增生症1例

患者男,63岁。头皮、额部、双耳前至面颊部、两下颌部和胸背部暗红色浸润性丘疹、结节、斑块6年余,伴严重瘙痒。浅表淋巴结和胸部CT和腹部彩超均未见异常。皮损组织病理示:真皮大量单个核细胞弥漫浸润,部分细胞体积较大,胞质淡,核椭圆形,可见核沟。免疫组织化学检查示:CD1a及S-100染色阳性。诊断:皮肤朗格汉斯细胞组织细胞增生症。     

婴幼儿Kaposi水痘样疹2例并文献复习

例1女,头面部起疹伴痒4个月,加重伴起水疱、发热3d。例2男,全身起疹3个月,头面部起水疱伴发热3d。均诊断为Kaposi水痘样疹。2例患儿均予更昔洛韦联合人免疫球蛋白治疗后痊愈。     

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。     

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。     

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.     

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。     

朗格汉斯细胞组织细胞增生症中TP53蛋白表达及基因突变检测

目的检测朗格汉斯细胞组织细胞增生症(LCH)中TP53蛋白表达和TP53基因全外显子突变,以探讨TP53在LCH发生发展中的作用机制。方法应用免疫组化SP法检测38例LCH中TP53蛋白水平;对16例TP53蛋白阳性组织原位提取DNA后PCR直接测序检测TP53基因外显子2～11的变化。结果 38例LCH样本TP53蛋白阳性16例(42.11%)。在16例阳性的平均吸光度比较中,分别为10例广泛型(MS)(0.1206±0.0088),6例局限型(SS)(0.1221±0.0030),二者表达差异无统计学意义(P>0.05)。PCR直接测序16例阳性LCH,2～11号外显子突变未见异常。TP53codon72基因型频率为:LCH组PRO型3例,ARG型5例,PRO/ARG型8例;与40例正常对照相比较分布差异无统计学意义(P>0.05)。LCH组中MS组PRO型2例,ARG型3例,PRO/ARG型5例;SS组PRO型1例,ARG型2例,PRO/ARG型3例。两组频率分布差异无统计学意义(P>0.05)。结论 LCH中有朗格汉斯细胞组织细胞TP53蛋白异常表达,但在临床分型上比较差异无统计学意义;LCH中TP53基因在全外显子无突变。TP53codon72基因型与正常人群一致且与临床分型及预后无相关。在LCH中可能有另一无关基因突变的机制影响了TP53蛋白表达。     

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。     

先天自愈性朗格汉斯组织细胞增生症一例

患儿,女,1个月。头面部红色丘疹、结痂,逐渐增多1个月。皮肤科查体：头皮、额头、躯干、四肢散在黄豆至豌豆大红褐色至黄褐色丘疹、丘疱疹、结痂,部分皮疹少量鳞屑,不易刮除。皮损组织病理示：表皮细胞间水肿,单一核细胞移入,基底细胞液化变性,真皮浅中层较多单一核细胞浸润,细胞核大、胞浆丰富。DIF:表皮细胞间及基底膜带IgG、C3、IgM、IgA阴性。免疫组化：CD1a、S100、CD68弱阳性,Langerin+。未予治疗,随访1个月皮疹基本消退,无新发皮疹。     

Purpuric nail striae as the initial manifestation in multisystem Langerhans cell histiocytosis

Nail involvement in Langerhans cell histiocytosis (LCH) is very uncommon. We describe a 9-month-old boy with longitudinal purpuric bands, onychodystrophy, and paronychias on his fingernails and toenails, which antedated manifestations of recurrent bilateral spontaneous pneumothorax and elevated transaminases. The diagnosis of nail LCH was then confirmed by immunohistochemistry demonstrating positive S100 and CD1a staining in the epithelium of nail bed tissue. This case report affirms that nail involvement may be the initial manifestation of multisystem LCH and could portend an unfavorable prognosis.     

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.     

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。     

Langerhans cell hyperplasia in the tumor stage of mycosis fungoides: a mimic of Langerhans cell histiocytosis

Mycosis fungoides is a form of cutaneous T-cell lymphoma (CTCL). Malignant CD4⁺ T cells have been found to adopt the T-regulatory (Treg) cell phenotype and function. We present the case of a 66-year-old man diagnosed with mycosis fungoides that was progressing from the plaque to the tumor stage. The histopathological examinations showed that the Langerhans cell (LC) infiltration in the skin lesion of the tumor stage was greater than that in the patch/plaque stage; the tumor stage lesions resembled LC histiocytosis pathologically. The associations among LCs, apoptotic tumor cells, Treg CTCL cells, and relevant cytokines are complex. Treg CTCL cells produce the immunosuppressive cytokines interleukin-10 and transforming growth factor beta, which facilitate continuous recruitment of LCs and maintenance of long-term dendritic cell immaturity, thereby explaining the remarkable LC infiltration in the tumor stage samples from our patient. This phenomenon indicates that LCs accompanied by Treg CTCL cells may play an important synergistic role in the tumor progression. The development of immunotherapy directed against Treg CTCL cells and LCs overproduction and other immunosuppressive cytokines may be a potent useful adjuvant and worthy of further investigation.