Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule

Abstract

Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence.

Bone pathologic fracture revealing an unusual association: coexistence of Langerhans cell histiocytosis with Rosai-Dorfman disease

“…Cells with emperipolesis have typically RD-cells immunophenotype, especially negative for CD1a and langerin (CD207). When combined in the same organ, the RDD component seems to be larger than the LCH component, that presents often as scattered CD1a + cells [4, 7, 13]. This habitual pattern is not seen at immunohistochemistry in our case.…”

“…The pediatric population is about one third of all cases (6 cases under the age of 18). In most cases, at initial presentation, the disease is limited to one organ (a total of 15 cases) : 9 cases in lymph nodes, 5 in the skin, 1 case in the bone (our current case) [4, 7–11, 13]. In fact, our patient is the only case among all reported cases to have a synchronous association of RDD-LCH in the bone.…”

“…The association between RDD and LCH is extremely rare, since the first case reported by Wang KH et al[7], 16 other additional cases have been reported in the english literature [4, 5, 7–13] (Table 1) through single-reported cases, except for the 9 reported cases (largest series) by O’Malley DP et al [4] and one case among a series of 25 cutaneous RDD in China, reported by Kong YY et al [8]. To the best of our knowledge, to date we have a total of 18 reported cases of association between RDD and LCH (RDD-LCH).…”

“…of casesAge (years)SexSiteWang KH et al [7]2002145FSkinKong YY et al [8]2007152FSkinSachdev R et al [9]200813MLNO’Malley DP et al [4]2010925 a 2 M/7 FLN : 8Abdominal mass/SC : 1Llamas-Velasco M et al [10]2012168MSkinCohen-Barak. E et al [5]2014110MSkin-BoneJin W et al [11]2014120FSkinKutty SA et al [12]2015131MBone-dura materLitzner BR et al [13]2015148FSkinOur case2016130FBone a the average age of the 9 reported cases; F female sex, M male sex, LN lymph node, SC subcutaneous …”

BRAF-V600E–mutated Rosai-Dorfman-Destombes disease and Langerhans cell histiocytosis with response to BRAF inhibitor

“…Langerhans cell histiocytosis (LCH) and Rosai-Dorfman-Destombes disease (RDD) exist in the L-group and R-group, respectively, based on recent reclassification of histocytoses. 2 Although there are a handful of mixed RDD/LCH reports (unassociated with other neoplasia), [3][4][5][6][7][8][9][10][11] the concept of "mixed histiocytosis" has varied definitions, including lesions either simultaneously occurring in the same location with distinct microenvironments vs those lesions in the same patient at different time points.…”