Cutaneous Rosai-Dorfman Disease With Increased Number of Eosinophils: Coincidence or Histologic Variant?

Abstract

N Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.

Rosai–Dorfman‐type histiocytes occur in eosinophilic chronic rhinosinusitis and potentially play a role in disease initiation and persistence

“…Indeed S100 is a non‐specific marker of dendritic cells, and dendritic cell subsets have been shown to be increased, and their ratios altered, in severe CRS with nasal polyps . The presence of eosinophils and in conjunction with RDD has been reported in association with idiopathic hypereosinophilic syndrome at autopsy in multiple sites, and in a patient with known RDD who later developed cutaneous lesions . Eosinophilic emperipolesis has been described recently in a case of nodal Langerhans cell histiocytosis .…”

“…22,23 The presence of eosinophils and in conjunction with RDD has been reported in association with idiopathic hypereosinophilic syndrome at autopsy in multiple sites, 24 and in a patient with known RDD who later developed cutaneous lesions. 25 Eosinophilic emperipolesis has been described recently in a case of nodal Langerhans cell histiocytosis. 26 No reports were found documenting the presence of eosinophilic emperipolesis in RDD, which was a constant feature in all of our cases.…”

Erdheim‐Chester disease with prominent pericardial effusion

“…Coexistent ECD and LCH have been documented and should be suspected in patients with atypical presentation of either disorder . Another similar lesion is RDD . Primarily a histopathologic diagnosis, RDD is characterized by the presence of a large number of histiocytes, many of which contain engulfed inflammatory cells (emperipolesis), and admixed lymphocytes, neutrophils and plasma cells in various proportions .…”

“…Another similar lesion is RDD . Primarily a histopathologic diagnosis, RDD is characterized by the presence of a large number of histiocytes, many of which contain engulfed inflammatory cells (emperipolesis), and admixed lymphocytes, neutrophils and plasma cells in various proportions . Eosinophils are considered uncharacteristic of the disease, although rare cases with prominent eosinophilic infiltrates have been documented .…”

Cutaneous Hematopathology

“…A xanthomatous component has been described in a subset of cases (Thawerani et al 1978 ;Quaglino et al 1998 ), as well as coexisting foci of Langerhans cell histiocytosis (LCH) (Wang et al 2002(Wang et al , 2006Kong et al 2007 ). Eosinophils are rare except in the foci of LCH (Cangelosi et al 2011 ). Many cases show various degrees of stromal fi brosis with or without a storiform pattern (Kong et al 2007 ;Kuo et al 2009 ).…”