Rosai Dorfman disease with nodal and multiple bone involvement: case report

Enfermedad de Rosai Dorfman con compromiso ganglionar y óseo múltiple: reporte de caso

Introduction: Rosai-Dorfman disease is a rare form of sinus histiocytosis, characterized by massive infiltration of histiocytes in lymph nodes and extranodal sites. Clinically, it usually presents with massive lymphadenopathy, fever, elevated erythrocyte sedimentation rate (ESR), and hypergammaglobulinemia. Treatment may be conservative or include corticosteroids, antimetabolites, or surgery in specific cases. Objective: To present a case of Rosai-Dorfman disease with multiple lymph node and bone involvement in a pediatric patient, highlighting that the primary care physician often plays a key role in evaluating the initial clinical syndrome and referring the patient for specialized diagnosis and management. Clinical Case: A 7-year-old female patient presented with bilateral cervical and inguinal adenopathy, intermittent fever, night sweats, and leg pain. Radiographs reported multiple polyostotic lytic lesions in the pelvis and appendicular skeleton. Differential diagnoses included Langerhans cell histiocytosis, disseminated histoplasmosis, and Hodgkin lymphoma. Biopsy of a lesion in the left ulna confirmed the diagnosis of Rosai-Dorfman disease. Conclusions: The nonspecific presentation of this disease and its similarities to other conditions underscore the importance of an interdisciplinary approach and a broad differential diagnosis. Recognizing its features aids in achieving an accurate diagnosis and appropriate treatment.