A Rare Case of Benign Histiocytic Neoplasm of Cutaneous Rosai–Dorfman Disease: A Case Report and Literature Review

Abstract

Rosai–Dorfman disease (RDD) is a rare disorder characterized by the proliferation of histiocytes in lymph nodes. It can occur anywhere in the body but commonly involves the cervical area of the neck. Its clinical presentation varies and patients with skin manifestations may develop papules, nodules, plaques, or pustules. Histologically, it typically presents with emperipolesis, where intact lymphocytes are found within histiocytes. The definitive treatment of RDD is not well established given the rarity of the disease and indeed skin lesions can regress spontaneously. Therapeutic treatment options include cryotherapy, radiation, or topical agents such as steroids or retinoids. Here we describe the case of a 24-year-old Hispanic female who presented with skin manifestations which proved to be histologically positive for Rosai–Dorfman disease. The patient clinically improved following the administration of intralesional steroids.

Diagnostic consideration of lipoma-like lesion: A case report of primary cutaneous Rosai-Dorfman disease

“…The histological signature common to both systemic and primary cutaneous Rosai-Dorfman disease is characterized by the proliferation of polygonal histiocytes exhibiting distinctive emperipolesis, wherein the cytoplasm of histiocytes appears to engulf lymphocytes, red blood cells, and/or granulocytes [ 2 , 3 , 8 ]. Additionally, mixed inflammatory infiltrates containing histiocytes, lymphocytes, and plasma cells are observed [ 2 , 8 , 10 ]. Immunohistochemically, histiocytes in these lesions typically demonstrate positive staining for S-100 and CD68, while CD1a shows negative staining [ 2 , 8 ].…”

“…In the context of our specific case, surgical excision proves particularly relevant. For lesions presenting in vital organ involvement or extranodal disease that is amenable to resection, this approach becomes a pivotal component of the therapeutic plan [ 4 , 10 ]. The 80 % reported cure rate reinforces the significance of surgical excision in achieving favorable outcomes, and its application in our case could contribute to both symptomatic relief and the potential for a successful resolution of the cutaneous variant of Rosai-Dorfman Disease [ 4 ].…”