Journal des rapports de cas cliniques

Rosai-Dorfman Disease in a 23-Year-Old Patient

Abstract

Hela Hariz, Sameh Marzouk, Raida Ben Salah, Noura Saidi, Moez Jallouli, Tahia Boudawara and Zouhir Bahloul

Rosai-Dorfman Disease (RDD) also known as Sinus Histiocytosis With Massive Lymphadenopathy (SHML) is a rare and benign non- Langerhans histiocytosis. First described in 1965 by Destombes [1] then in 1969 by Rosai and Dorfman, this disease is characterized by benign histiocytic proliferation with frequent lymphophagocytosis (emperipolesis) [2]. All ages are affected but most of the patients present before age 20, with a slight male predominance [3]. Rosai-Dorfman disease usually presents as bilateral, massive and painless cervical lymphadenopathy [2]. Extra-nodal involvement is found in 30-40% of cases and is more common in the head and neck region [3]. The disease is often benign and self limiting requiring only observation. In some cases, the condition can be severe. Several treatments have been used with varying efficiency [4]. We report a case of RDD in a male Tunisian patient treated and followed up in our department.