皮肤巨淋巴结病性窦组织细胞增生症

报告1例皮肤巨淋巴结病性窦组织细胞增生症。患者女,47岁。面颈部无痛性棕黄色斑块3年,无淋巴结增大及其他系统受累。皮损组织病理检查显示真皮全层及皮下组织中有大量组织细胞浸润,其间有淋巴细胞及浆细胞,并可见组织细胞内吞噬淋巴细胞及浆细胞。免疫组化染色结果示组织细胞S-100蛋白、DC68均阳性。皮损组织分枝杆菌PCR扩增、分枝杆菌培养及真菌培养均为阴性。诊断：皮肤巨淋巴结病性窦组织细胞增生症。     

糖皮质激素联合羟氯喹治疗皮肤型Rosai-Dorfman病1例

<正>Rosai-Dorfman病(RDD)即窦性组织细胞增生病伴巨大淋巴结病,是一种罕见的良性自限性疾病,偶可侵袭进展,死亡率高~[1]。罕见情况下,RDD可仅有皮肤而无淋巴结累及或其他系统异常即皮肤窦性组织细胞增生病(cuta-neous Rosai-Dorfman disease,CDRR)~[2]。现将笔者诊治的1例报告如下。1病历摘要患者男,57岁。左侧面部发生暗红色结节、斑块8个月,患者自述8个月前局部被蚊虫叮咬后出现红色     

皮肤窦组织细胞增生症伴巨大淋巴结病1例

报告1例窦组织细胞增生症伴巨大淋巴结病。患者,男,83岁。右面部暗红色斑块、斑丘疹1年,无淋巴结增大及系统受累。皮损组织病理检查示真皮内大量组织细胞增生。免疫组化染色示S-100蛋白及CD68阳性,CD1a阴性。依据临床表现、组织病理改变和免疫组化检查,确诊为窦组织细胞增生伴巨大淋巴结病。     

皮肤窦性组织细胞增生症1例

皮肤窦性组织细胞增生症(Rosai-Dorfman病),是一种无系统损害的罕见病例。本例皮损表现为黄红色丘疹、斑块和结节,无全身淋巴结受累。组织病理示:真皮层组织细胞增生结节,可见淋巴细胞吞噬现象。免疫组化示:病变组织细胞S-100(2+)、CD68(2+)、CD1a(-)、Ki-67(-)。     

皮肤Rosai-Dorfman病1例

患者女,44岁。面部、右下肢出现红斑、结节1个月,无淋巴结增大,无系统受累。皮损组织病理示:真皮内多量组织细胞样细胞浸润。免疫组织化学染色示:S-100蛋白(+),CD68(+),CD1a(-)。诊断:皮肤Rosai-Dorfman病。     

皮肤窦性组织细胞增生症一例并文献复习

报道1例皮肤窦性组织细胞增生症并对文献进行复习。患者,女,56岁,右上肢暗红色结节、斑块2年余,无系统受累。组织病理：真皮内大量组织细胞、淋巴细胞,可见组织细胞伸入现象。免疫组化：S-100（+）、CD68（+）、CD1a（-）。外用卤米松等治疗后皮损范围已明显缩小。     

皮肤窦组织细胞增生症伴巨大淋巴结病的研究进展

窦组织细胞增生症伴巨大淋巴结病是一种病因不明的良性组织细胞增生性疾病,又称Rosai-Dorfman病.可累及多个器官,临床表现为发热、颈部淋巴结肿大、皮肤斑块、结节,伴有白细胞增多、高γ球蛋白血症、血沉增快.仅有皮肤损害,无其他器官受累者称为皮肤Rosai-Dorfman病.皮损组织病理可见真皮大量组织细胞浸润,在组织细胞的胞浆内可见被吞噬的完整的淋巴细胞、嗜中性粒细胞等.部分患者皮疹可自行消退,治疗可采用手术切除、口服糖皮质激素、反应停、维甲酸等.     

进行性结节性组织细胞增生症

报告1例进行性结节性组织细胞增生症。患者女,22岁。全身皮肤丘疹和结节6年,皮损进行增多和增大,无自行消退倾向,血脂正常范围,皮损组织病理改变符合梭形细胞性黄色肉芽肿。临床表现结合组织病理改变诊断进行性结节性组织细胞增生症。复习英文文献,至今共有18例报道,该例显著特点是出现黏膜及眼睛受累,免疫组化S-100蛋白阳性在进行性结节性组织细胞增生症中较为少见。     

原发性皮肤Rosai-Dorfman病1例

Rosai-Dorfman病(Rosai-Dorfman's Disease, RDD)最常见的症状是颈部淋巴结肿大,又名窦性组织细胞增生症伴巨大淋巴结病(Sinus histiocytosis with massive lymphodenopathy),仅10%的病人伴有皮损.如果原发于皮肤而不伴淋巴结病者称为原发性皮肤RDD,临床十分罕见1.     

窦组织细胞增生伴巨大淋巴结病

报告1例窦组织细胞增生伴巨大淋巴结病.患者男,27岁.头皮、颈部、躯干部丘疹及结节半年,伴双侧颌下淋巴结肿大3个月.皮损组织病理检查示真皮内大量组织细胞增生,可见伸入运动.淋巴结组织病理检查示淋巴窦明显扩张,窦内可见大量组织细胞及伸人运动.免疫组化染色示S-100蛋白及CD68阳性,CD1a阴性.依据临床表现、组织病理改变和免疫组化检查,确诊为窦组织细胞增生伴巨大淋巴结病.     

Solitary nodule of the penis as unique manifestation of Rosai-Dorfman disease

We report the case of a 53-year-old man with a solitary nodule located on the inner surface of the prepuce. The lesion was removed and the final diagnosis was Rosai-Dorfman disease (RDD/sinus histiocytosis with massive lymphadenopathy). This report, being the first case of RDD presenting as a single nodule of the penis, broadens the spectrum of the cutaneous expression of RDD.     

Coexistence of localized Langerhans cell histiocytosis and cutaneous Rosai-Dorfman disease

Rosai-Dorfman disease (RDD; sinus histiocytosis with massive lymphadenopathy) and Langerhans cell histiocytosis (LCH) are two different yet pathogenetically related histiocytic disorders. While systemic and localized forms have been identified in both diseases, each has its own characteristic histological, immunohistochemical and ultrastructural profile. Rarely, either RDD or LCH can also occur in the context of certain malignant neoplasms. However, the coexistence of RDD and LCH has never been described. We report a case of cutaneous RDD in which a focus of LCH was found. Clinical and laboratory examinations revealed no evidence of extracutaneous involvement of RDD or LCH. We believe that this is the first report of such a coexistence, and the possible pathogenesis is discussed.     

累及皮肤、眼、骨骼的Rosai-Dorfman病一例并文献复习

Rosai-Dorfman病(RDD)可累及皮肤、腮腺、眼睑、肝脏或乳房等,但同时累及皮肤、眼、骨骼的RDD尚未见报道。本文报道一例并复习相关文献。患者,男,41 岁。右侧面部出现麻木感3个月,后于相同部位出现紫红色丘疹1个月,此时无系统受累。组织病理检查：真皮内淋巴组织细胞、浆细胞、巨细胞、中性粒细胞浸润。免疫组化示：组织样细胞S100（＋）、CD68（＋）、CD1a（－）,诊断：皮肤Rosai-Dorfman病。患者口服沙利度胺及外用丁酸氢化可的松乳膏和他克莫司软膏治疗2个月后皮损消退,但7个月后皮下组织增生累及眼眶并破坏骨质,至眼科行手术治疗,目前仍在随访中。     

Rosai-Dorfman病的治疗新进展

【摘要】 Rosai-Dorfman病又称窦性组织细胞增生症伴巨大淋巴结病,是一种临床上罕见的病因不明的良性自限性组织细胞增生症。本病缺乏特异的治疗方法。治疗首选手术,无法进行手术的患者可以选用局部治疗和系统治疗。局部治疗包括外用糖皮质激素或局部封闭治疗,光动力治疗和激光免疫疗法也是很有前景的局部疗法。传统的系统治疗包括糖皮质激素、免疫调节剂、阿维A等;对CD20阳性的Rosai-Dorfman病,利妥昔单抗是一种新的治疗选择。本文就近年来Rosai-Dorfman病的一些治疗新进展予以综述。     

Five cases of cutaneous Rosai-Dorfman disease

Rosai-Dorfman disease (RDD), previously known as sinus histiocytosis with massive lymphadenopathy, is a benign, idiopathic histiocytic proliferative disorder. It commonly affects lymph nodes, but any organ of the body may be involved. Histological findings include characteristic large, pale, histiocytic cells (Rosai-Dorfman cells) exhibiting cytophagocytosis. Immunohistochemically, these histiocytes are positive for S-100 protein and CD68, but stain negatively for CD1a. On electron microscopy, Birbeck granules are absent. RDD limited to the skin is rare, less than 30 cases having been reported to date. We present five further cases of purely cutaneous RDD. Three presented as solitary nodules and one as a large, well-circumscribed plaque. The fifth patient, who was HIV positive, had a rosacea-like facial eruption.     

Rosai-Dorfman disease with cutaneous manifestation (sinus histiocytosis with massive lymphadenopathy)

We report a 73-year-old woman with the typical findings of "Rosai-Dorfman disease" (RDD) including massive, painless lymphadenopathy, fever and multiple, asymptomatic cutaneous lesions on her face, collum and neck. Histologically, the lesions were marked by characteristic large macrophages exhibiting emperipolesis.     

Cutaneous rosai-dorfman disease is a distinct clinical entity

Rosai-Dorfman disease (RDD) is a rare but distinctive clinicopathologic entity of unknown etiology affecting lymph nodes as well as extranodal sites. Although cutaneous involvement in RDD is common, purely cutaneous disease is rare and not well documented. We report 22 patients with cutaneous and superficial subcutaneous RDD. The lesions presented as papules and nodules, often with discoloration (9/22) and frequent multifocality (13/22), without predilection for a specific site of the body. Age distribution was wide and ranged from 15 to 68 years, with a median of 43.5 years. Of the 17 patients for whom information on racial background was available, 7 were Asian, 8 were white, and 2 were black, with a marked female predominance (2:1). The lesions resolved in 6 of 13 patients for whom follow-up data were available, regardless of the treatment given. Lesions persisted or recurred in 7 patients. Histologically, the lesions are invariably characterized by a proliferation of polygonal S100-positive histiocytes showing emperipolesis and a mixed inflammatory infiltrate. This study characterizes the histologic spectrum of cutaneous RDD in regard to variation in the numbers of typical S100-positive histiocytes and emperipolesis, variation in the quality and quantity of the inflammatory response, and the degree of stromal fibrosis, which resulted in a strikingly storiform growth pattern in six lesions and a lobulated pattern in two lesions. Whereas the clinical as well as histologic appearance of the cutaneous and subcutaneous lesions in the purely extranodal forms of RDD is indistinguishable from that of systemic RDD, this study emphasizes that purely cutaneous RDD is a distinct clinical entity in regard to its epidemiology and remains localized to the skin even with long-term follow-up. Patients with purely cutaneous RDD are of an older age at onset of disease (median = 43.5 years), with a reversed male/female ratio. There are no significant systemic extracutaneous or serologic manifestations. Whereas systemic RDD is commonly seen in blacks and only rarely reported in Orientals, the majority of the patients in this series with purely cutaneous RDD are Asians and whites.     

Successful treatment of Rosai–Dorfman disease with low-dose oral corticosteroid

We present herein a Japanese case of Rosai–Dorfman disease (RDD) in which cutaneous manifestations completely remitted after treatment with low-dose oral corticosteroid. A 69-year-old Japanese man presented with a 1-year history of enlarged submandibular lymph nodes and subsequent nasal and pharyngeal bleeding. RDD was diagnosed based on biopsy results from a lymph node in the left parotid region. The patient had also noted several skin eruptions that repeatedly appeared and disappeared on the face and arms. Biopsies were taken from skin eruptions on the face and cuboidal fossa. Both biopsy specimens showed dense, well-demarcated infiltration of histiocytes, lymphocytes and multinucleated giant cells from just under the epidermis to the subcutaneous tissue. These histiocytes were positive for CD68 and S-100, but negative for CD1a, and some displayed emperipolesis. Given the histopathological findings and the fact that the patient was suffering from RDD, skin lesions were diagnosed as cutaneous manifestations of RDD. Cutaneous lesions gradually began to persist concomitant with enlargement of extranodal lymphadenopathy in the nasopharyngeal area. Increasing respiratory obstruction prompted a trial with oral prednisolone commencing at 0.4 mg/kg per day. Both the lymphadenopathy and skin lesions responded quickly. Within 3 months, all his skin lesions disappeared completely with almost complete resolution of lymphadenopathy. Twelve months after the beginning of oral prednisolone therapy, slight recurrence of mucosal and cutaneous lesions appeared, but disappeared quickly with an increase in prednisolone to 0.3 mg/kg per day. Low-dose prednisolone appeared very effective in the case of RDD.     

Cutaneous Rosai-Dorfman disease: comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case

BACKGROUND: The latest comprehensive review of primary cutaneous Rosai-Dorfman disease was published as part of an exhaustive survey of sinus histiocytosis with massive lymphadenopathy in 1990. Since then, much progress has been made in the understanding of malignant lymphoma and benign disorders of lymphoid and histiocytic origin. OBJECTIVE: We reviewed cases of primary cutaneous Rosai-Dorfman disease published since 1990 and discuss their clinical and pathologic features, comparing them with cases of systemic Rosai-Dorfman disease. METHODS: We conducted a search of the National Library of Medicine PubMed database for cases of cutaneous Rosai-Dorfman disease reported in the English-language medical literature since February 1990. RESULTS: We identified 72 patients with cutaneous Rosai-Dorfman (female to male ratio 1:0.5). The gross appearance and number or distribution of lesions were highly variable. Abnormal laboratory data included peripheral blood cytopenias (10 patients) and increased gammaglobulin fraction (10 patients). The response to treatment was variable. CONCLUSION: Purely cutaneous disease without the characteristic lymphadenopathy is rare but has been increasingly reported in the literature. Compared with patients with systemic Rosai-Dorfman disease, patients with primary cutaneous Rosai-Dorfman disease are older, women are more commonly affected, and whites are more likely than blacks to be afflicted.     

Histologic features of cutaneous sinus histiocytosis (Rosai-Dorfman disease): study of cases both with and without systemic involvement

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a systemic proliferation of cells that resemble the sinus histiocytes of lymph nodes. Whereas initial reports highlighted the often striking cervical adenopathy, more than 40% of patients have extranodal involvement. Cutaneous lesions are the most common form of extranodal disease, but cases that present as cutaneous disease without lymph node involvement are rare. We examined biopsies from three patients with cutaneous lesions of sinus histiocytosis, none of whom had detectable systemic involvement, and compared them to those of two patients whose lymph nodes were involved by the disease. The histology of skin lesions in all five cases showed dense nodular or diffuse infiltrates of "histiocytes," emperipolesis of lymphocytes, neutrophils, and plasma cells. "Histiocytes" were present in lumens of dilated lymphatics. At the peripheries of the lesions were lymphoid aggregates and thick-walled vessels surrounded by plasma cells. Staining with anti-S-100 protein antibody showed marked cytoplasmic positivity in the "histiocytes" in each case. The only features that differed among the two groups were the presence of pseudoepitheliomatous hyperplasia and infiltrates of eosinophils in one case in which the disease was limited to the skin. We believe that cutaneous sinus histiocytosis can be specifically diagnosed by skin biopsy. Because cutaneous sinus histiocytosis may be unfamiliar to general pathologists, dermatopathologists, and dermatologists, cases limited to the skin may be under-recognized.