What is multicentric reticulohistiocytosis?
Multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans.
What is reticulohistiocytosis?
Reticulohistiocytosis is a rare form of histiocytosis that can affect the skin and other organs. Reticulohistiocytosis has a wide range of severity. It can be a limited disease with a single yellowish-red skin lump through to multiple skin lesions with internal organ involvement. It usually occurs in adults.
What causes histiocytosis?
The exact cause of histiocytosis is unknown. However, recent studies indicate that it is caused by the development and expansion of an abnormal Langerhans cell that subsequently leads to the accumulation of other cells of the immune system, resulting in collections or tumors in various areas of the body.
What causes erdheim Chester disease?
The exact cause of ECD is unknown. However, the disease is thought to represent an abnormal inflammatory process characterized by excessive proliferation and accumulation of certain cells, with associated scarring or overgrowth of fibrous connective tissue (fibrosis).
What is the difference between multifocal and multicentric?
The presence of two or more foci of cancer within the same breast quadrant is defined as multifocal, while the presence of two or more foci of cancer in different quadrants of the same breast is defined as multicentric.
What is multicentric breast disease?
A multicentric breast cancer is a term given to a breast cancer where there are two or more breast cancers separated by normal breast tissue (often taken as 5 cm of separation 4). It is related to but distinct from the term multifocal breast cancer.
Is histiocytosis curable?
Usually a cure is linked to being in remission for a certain period of time. There is no established period of “non-active” disease before LCH is considered cured, but the chance for recurrence is low after five years from end of treatment.
Is histiocytosis hereditary?
Langerhans cell histiocytosis is usually not inherited and typically occurs in people with no history of the disorder in their family. A few families with multiple cases of Langerhans cell histiocytosis have been identified, but the inheritance pattern is unknown.
Is erdheim-Chester hereditary?
It is not believed to be contagious or hereditary. The cause is not known. The first two cases of ECD were reported by scientists Jakob Erdheim and William Chester in 1930.
How common is Erdheim-Chester disease?
Erdheim-Chester disease is a rare disorder; its exact prevalence is unknown. More than 500 affected individuals worldwide have been described in the medical literature. For unknown reasons, men are slightly more likely to develop the disease, accounting for about 60 percent of cases.