Rosaï-Dorfman-Destombes Disease: A Clinical Case Report

Abstract

Rosaï-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare and benign histoproliferative disorder of unknown etiology. It commonly presents with bilateral, painless cervical lymphadenopathy accompanied by systemic features such as fever and leukocytosis. RDD manifests in two primary forms: a systemic form involving multiple organ systems and a cutaneous form confined to the skin. Histopathological examination typically reveals pericapsular fibrosis, sinusoidal dilation, and the presence of large histiocytes exhibiting emperipolesis (phagocytosed lymphocytes within histiocytes). Extranodal involvement is observed in approximately 43% of the cases, with a predilection for the head and neck regions. Although the precise etiology remains unclear, associations with viral infections such as Epstein-Barr virus (EBV) and human herpesvirus-6 (HHV-6) have been proposed. Management strategies are case-dependent, ranging from observation in cases of spontaneous remission (reported in 20-50% of patients) to more aggressive therapeutic interventions. Here, we describe a pediatric case of RDD managed in Morocco.