DOI: 10.36347/sjmcr.2021.v09i07.003

Pages: 699-701

Sinus Histiocytosis with massive lymphadenopathy (SHML) Rosai-Dorfman Disease is a rare, idiopathic, node-based histiocytic proliferative disorder affecting different age group, 10% of the incidence in children less than 10 years old and 80% in young age group less than 20 years old [1]. Higher incidence of the disease was reported in African and West Indian descent [1]. The disease usually presents with massive painless cervical lymphadenopathy as part of a generalized process involving lymph nodes and may involve extranodal sites independently of lymph node status. The head and neck region represents one of more common extranodal areas, particularly the Sinonasal tract [1]. Here we report the first presentation of 7 years old child with unusual presentation of sinus histiocytosis (Rosai –dorfman disease) and hepatospleenomegly in Sudan.