Rosai-Dorfman-Destombes disease with renal involvement and secondary glomerulopathy: Report of an exceptional case

Rosai-Dorfman-Destombes disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare disorder, first described by Destombes in 1965 and then Rosai and Dorfman reported the first clinical series in 1969. The etiology of RDD is unknown, and the treatment is not standardized. There is no data about the true number of cases that exist worldwide. This disease occurs mainly in children and young adults, however, it may occur at any age. It is more common in males and individuals of African descent. RDD, presents clinically as painful bilateral massive cervical lymphadenopathies, with fever, night sweats and weight loss. Mediastinum, inguinal region and retroperitoneum lymph nodes may also be involved. The course of the disease is unpredictable, usually characterized by episodes of exacerbation and remission. It can last for many years and is usually self-limited, with good outcomes. Extranodal compromise is unusual but can affect almost any organ. Involvement of the kidney has been stated as a poor prognostic predictor.1 We present an unusual case of RDD in a 64 year old woman with a literature review of renal RDD.