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. LEARNING POINTS RDD is a rare self-limiting benign lymphoproliferative disorder, which can be associated with autoimmune disease, hematological malignancies, and post-infectious conditions.Skin lesions can be self-limiting in many cases of RDD.Therapeutic treatment options include cryotherapy, local radiation, and topical steroids and retinoids.