What is the prevalence of Erdheim Chester Disease?

Erdheim-Chester Disease (ECD) is an extremely rare form of non-Langerhans cell histiocytosis, characterized by the excessive production and accumulation of certain white blood cells called histiocytes. Due to its rarity, the prevalence of ECD is not well-established. However, it is estimated that ECD affects approximately 1 in every 200,000 to 1 in every 500,000 individuals worldwide. The disease primarily affects adults, with a median age of diagnosis around 53 years old. ECD is slightly more common in males than females.

Although ECD can occur in any ethnic group, it has been reported more frequently in individuals of European descent. The disease often presents with a wide range of symptoms, including bone pain, fatigue, weight loss, and neurological abnormalities. ECD can affect multiple organs, such as the bones, heart, lungs, kidneys, and brain. The diagnosis of ECD can be challenging due to its rarity and the similarity of symptoms to other conditions.

While ECD is considered a rare disease, ongoing research and increased awareness have led to improved diagnosis and understanding of the condition. Collaborative efforts among medical professionals and researchers aim to further elucidate the prevalence and underlying mechanisms of Erdheim-Chester Disease.