Cutaneous Rosai-Dorfman Disease Is a Distinct Clinical Entity

Abstract

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.

Rosai-Dorfman Disease Presenting as Isolated Subglottic Mass- an Extremely Rare Presentation

“…2 The extranodal sites include skin, soft tissues, respiratory system, genitourinary system, bones, CNS, orbit, thyroid and breast [2][3][4][5][6] in about quarter of patients isolated extra-nodal disease occurs. It can occur in children and in adults and mean age of incidence is 20.6years with Male to female ratio is 1.4:1.…”

“…It can occur in children and in adults and mean age of incidence is 20.6years with Male to female ratio is 1.4:1. 2,3 Patients with soft-tissue involvement tend to be older than patients with other extra-nodal sites. 3 The clinical & radiological features may vary depending on the site involvement.…”

“…2,3 Patients with soft-tissue involvement tend to be older than patients with other extra-nodal sites. 3 The clinical & radiological features may vary depending on the site involvement. The radiological imaging is confounding because of the broad differential diagnostic possibilities and biopsy of the mass is highly recommended.…”

“…2 The common extra-nodal sites are skin, eye, paranasal sinuses, genitourinary system, CNS, bone, breast, soft tissues and thyroid. [2][3][4][5][6] We are describing a case of Rosai-Dorfman disease presenting as isolated subglottic Mass with the chief complaint of progressive dyspnea.…”

Doença de Rosai-Dorfman cutânea: relato de caso

“…2,6 Purely extranodal cutaneous forms have already been reported; however, they occur rarely, with only 85 cases having been described up to 2006 in reports or short series, thus justifying the present case report. [10][11][12][13][14][15] …”