Histological variability and the importance of clinicopathological correlation in cutaneous Rosai-Dorfman disease

Abstract

Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The disease mainly affects lymph node tissue, although it is rarely confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her left leg, and sparse erythematous papules on her face and arms. A complete clinical response was achieved with thalidomide, followed by recurrence at the initial site one year later. The histological examination displayed the typical features of Rosai-Dorfman disease in the recent lesions but not in the older lesions. In the setting of no lymphadenopathy, the histopathological features of Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the histological aspects present at different stages, not always featuring the hallmark microscopic signs of Rosai-Dorfman disease, is particularly important for a correct diagnosis of this rare disorder.

Rosai-Dorfman Disease: Self-Resolving Unilateral Lymphadenopathy and a Brief Review of Literature

“…According to recent studies, cutaneous Rosai-Dorfman disease (CRDD) has been proposed as a distinct disease from RDD due to several pathological differences [ 6 , 12 ]. Such differences include exclusive involvement of the skin with possible nodal involvement in CRDD [ 12 ].…”

“…According to recent studies, cutaneous Rosai-Dorfman disease (CRDD) has been proposed as a distinct disease from RDD due to several pathological differences [ 6 , 12 ]. Such differences include exclusive involvement of the skin with possible nodal involvement in CRDD [ 12 ]. Demographic features differ considerably with the average age of CRDD diagnosis at 45 years and a predisposition among females, whereas the average age of the diagnosis of RDD is 20 years with a male predominance [ 4 , 6 – 8 ].…”

“…RDD has an increased incidence among African and West Indian populations [ 6 – 8 ]. Finally, CRDD is associated with a better prognosis compared to RDD [ 12 ]. The absence of coalescing pigmented papulonodular clusters, verrucous plaques, nodules, or satellite lesions ruled out a diagnosis of cutaneous Rosai-Dorfman disease (CRDD) in this case [ 13 ].…”

Emperipolesis and S100 expression may be seen in cutaneous xanthogranulomas: A multi‐institutional observation

“…Cutaneous manifestations of RDD can be clinically and histologically challenging to distinguish from XG in cases that lack the classic presentation of bilateral cervical lymphadenopathy. In skin‐limited RDD, dermatopathologists rely on the presence of emperipolesis, defined as intact inflammatory cells within the cytoplasm of another cell, in order to differentiate RDD from other histiocytic disorders . Immunohistochemical stains play an important role in aiding dermatopathologists in differentiating these diseases, as XG is comprised of CD68‐positive histiocytes that are negative for CD1a and S100, while RDD is usually positive for CD68 and S100, and negative for CD1a .…”

“…[3][4][5][6][7] Immunohistochemical stains play an important role in aiding dermatopathologists in differentiating these diseases, as XG is comprised of CD68-positive histiocytes that are negative for CD1a and S100, while RDD is usually positive for CD68 and S100, and negative for CD1a. 4,8 Tissue immunostaining is not absolute, however, as at least focal S100 positive staining has been described in some cases of XG. 9 Emperipolesis is not limited to RDD and can be identified in other settings, particularly in bone marrow biopsies of hematologic diseases including myeloproliferative disorders, non-Hodgkin lymphoma and idiopathic thrombocytopenic purpura.…”