Brenn Thomas, Calonje Eduardo, Granter Scott R, Leonard Niamh, Grayson Wayne, Fletcher Christopher D M, McKee Phillip H
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Am J Dermatopathol. 2002 Oct;24(5):385-91. doi: 10.1097/00000372-200210000-00001.
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.
罗萨伊 - 多夫曼病(RDD)是一种病因不明的罕见但独特的临床病理实体,可累及淋巴结及结外部位。虽然RDD累及皮肤很常见,但单纯皮肤型疾病罕见且记录不详。我们报告了22例皮肤及浅表皮下RDD患者。皮损表现为丘疹和结节,常伴有色素沉着(9/22)且多灶性常见(13/22),无身体特定部位的偏好。年龄分布广泛,为15至68岁,中位数为43.5岁。在可获取种族背景信息的17例患者中,7例为亚洲人,8例为白人,2例为黑人,女性明显占优势(2:1)。在有随访数据的13例患者中,6例皮损消退,无论接受何种治疗。7例患者皮损持续存在或复发。组织学上,皮损总是以多边形S100阳性组织细胞增生为特征,可见吞噬现象及混合性炎症浸润。本研究描述了皮肤RDD的组织学谱,涉及典型S100阳性组织细胞数量及吞噬现象的变化、炎症反应的质和量的变化以及间质纤维化程度,这导致6个皮损呈现显著的车辐状生长模式,2个皮损呈现分叶状模式。虽然单纯结外形式的皮肤及皮下RDD的临床及组织学表现与系统性RDD难以区分,但本研究强调,单纯皮肤型RDD在流行病学方面是一个独特的临床实体,即使长期随访也局限于皮肤。单纯皮肤型RDD患者发病年龄较大(中位数 = 43.5岁),男女比例倒置。无明显的全身皮肤外或血清学表现。虽然系统性RDD常见于黑人,在东方人中仅罕见报道,但本系列中大多数单纯皮肤型RDD患者为亚洲人和白人。