What is the prevalence of Rosai-Dorfman disease?
How many people does Rosai-Dorfman disease affect? Does it have the same prevalence in men and women? And in the different countries?
Rosai-Dorfman disease is a rare noncancerous disorder characterized by the overproduction and accumulation of immune cells called histiocytes. It primarily affects children and young adults, with a slight male predominance. The exact prevalence of this condition is unknown, but it is considered extremely rare. Rosai-Dorfman disease has been reported worldwide, but most cases have been documented in North America and Europe. Due to its rarity, the prevalence of this disease is difficult to determine accurately.
Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare non-cancerous disorder characterized by the overproduction and accumulation of a specific type of immune cells called histiocytes. While the exact prevalence of this condition is not well-established, it is considered extremely rare.
Due to its rarity, there is limited data available regarding the prevalence of Rosai-Dorfman disease. However, it is estimated to affect less than 1 in 200,000 individuals worldwide. The condition can occur at any age but is most commonly diagnosed in children and young adults, with a slight male predominance.
Rosai-Dorfman disease typically presents with painless, enlarged lymph nodes in the neck, although it can also affect other areas of the body. Additional symptoms may include fever, night sweats, weight loss, and fatigue. In some cases, the disease can involve extranodal sites such as the skin, eyes, bones, or central nervous system.
Diagnosis of Rosai-Dorfman disease involves a combination of clinical evaluation, imaging studies, and histopathological examination of affected tissues. Treatment options vary depending on the extent and severity of the disease, but may include observation, corticosteroids, surgical excision, or other immunosuppressive therapies.
While Rosai-Dorfman disease is a rare condition, it is important for healthcare professionals to be aware of its existence and consider it in the differential diagnosis of lymphadenopathy and related symptoms.
