Rosai dorfman syndrome with extranodal presentation

Abstract

Rosai-Dorfman disease (RDD) also known as sinus histiocytosis with massive lymphadenopathy is a rare idiopathic and non-neoplastic disorder of histiocytes, resembling the sinus histiocytes of lymph nodes. The most common presentation of RDD is painless cervical lymphadenopathy in a young patient. The typical clinical features of this disease include bilateral painless lymphadenopathy, fever, and polyclonal hyperglobulinemia. However, in about 25% of cases there is no lymph node involvement and the disease presents as a mass in skin, soft-tissue, nasal cavity, eye, bone, or in other extranodal sites (extranodal type). The condition may present with extranodal involvement in 43% of cases, and cutaneous lesions are the most common form of extranodal disease. This can pose a diagnostic challenge if RDD is not considered in differential diagnoses. Isolated extranodal RDD is relatively uncommon. We report this rare case of extranodal RDD in a 32-year-old female who presented with erythematous tender subcutaneous plaques on lower extremities with left periorbital swelling. Histopathological examination demonstrated lymphophagocytosis (emperipolesis) consistent with a diagnosis of RDD.