A Rare Case of Rosai Dorfman Destombes Disease Presenting With Lupus Nephritis and Central Nervous System Small Vessel Vasculitis

Abstract

Rosai-Dorfman-Destombes (RDD) disease is a rare syndrome characterised by benign lymphoproliferative disorder with sinus histiocytosis that presents with massive lymphadenopathy. It occurs mainly in children and young adults. It can be associated with autoimmune diseases like systemic lupus erythematosus (SLE). We present a 15-year-old male case of RDD who was diagnosed with SLE with class IV lupus nephritis and central nervous system small vessel vasculitis. Sensorium improved and lymph node size decreased with steroids. Hence, SLE can present with nephrito-nephrotic syndrome and central nervous system (CNS) small vessel vasculitis without any other systemic involvement. Steroids can be helpful in the management of RDD. Therefore, we should consider the possibility of RDD in cases of SLE with generalised lymphadenopathy, even though the association is rare.

Keywords: rdd; rosai-dorfman disease. sinus histiocytosis with massive lymphadenopathy. destombes–rosai–dorfman disease. spine. magnetic resonance imaging. computed tomography; sle and lupus nephritis; small vessel vasculitis; systemic steroids.