Rosai-Dorfman Disease Presented as Generalized Lymphadenopathy: A Case Report

Rosai-Dorfman disease (RDD) is an uncommon, histiocytic lesion that presents most commonly in young patients as large, painless cervical lymphadenopathy but can present with generalized lymphadenopathy without cervical LN involvement. It is characterized by the overproduction and accumulation of histiocytes, primarily in the lymph nodes, but extranodal involvement may also occur. Tissue biopsy is the main diagnostic modality. Histopathology is required to confirm the diagnosis of RDD. The clinical course is unpredictable regardless of treatment. Although in most cases RDD can be observed or treated with local therapies, some patients with refractory or multifocal disease experience morbidity and mortality. Considering the rarity of RDD presenting without cervical lymphadenopathy, we would like to report this case for the world, and up to our knowledge, it is the first case to be reported in our country. Here, we report a rare case of RDD in a 28-year-old male patient who presented with generalized lymphadenopathy over bilateral axillary, epitrochlear, and inguinal areas for the past year, which was histomorphologically confirmed to be RDD. He was treated with a steroid and showed a partial response to the treatment. RDD is a rare entity that is challenging in diagnosis and management. Clinicians and pathologists should have a high degree of suspicion for RDD in young patients with generalized lymphadenopathy, even with no cervical lymph node involvement. FNAC is a very important noninvasive and cost-effective modality to rule out top clinical differentials of RDD like lymphomas.