What is Lymphangiomatosis? Lymphangiomatosis has been characterized as a rare pathogenic condition in which multiple lymphangiomas appear in various body constituents and can involve the skeletal system, connective tissue, and visceral organs which can obstruct, compress, or destroy vital structures. Although considered benign and not metastatic, lymphangiomatosis can be persistently invasive, making treatment challenging and problematic. There is no standard definition describing this disease that has been agreed to by the medical/scientific community in part because the disease is quite rare, in part because the lymphatic system is understudied - thus poorly understood. Astonishing, but regrettably true. The cause, or etiology of lymphangiomatosis is unknown. It has been presumed to be caused by a multi-focal malformation of the lymphatic system - alone, or combined with malformation of the vascular system. Lymphangiomatosis can occur at any age, but the incidence is highest in infants and young children often with poor prognosis. http://lgdalliance.org/aboutlymph.asp
What is Gorham's disease? Gorham's disease is a rare musculoskeletal condition in which spontaneous, progressive resorption of the bone occurs. It is known variously as massive osteolysis, disappearing bone, or vanishing bone disease. Although described long ago, this rare and potentially catastrophic disease remains understudied, thus, poorly understood. It is thought to be closely related to, or even a severe form of, lymphangiomatosis. It may be characterized by a proliferation of thin-walled vascular capillaries or lymphatic vessels that starts within the bone. As these vessels proliferate, they aggressively invade the adjacent bone leading to resorption and replacement of angiomatous tissue. The cause remains unknown and it is not usually recognized by clinicians until a fracture occurs, with subsequent improper bone healing. Gorham's disease is extremely rare and may occur at any age. It is most often recognized in children and young adults, without gender, race predilection, or inheritance pattern. Because it is so rare, and commonly misdiagnosed, it is not known exactly how many people are affected by this disease. http://lgdalliance.org/aboutgorham.asp
All interventions (pharmacological and surgical) for either lymphangiomatosis or Gorhams disease are all still considered to be experimental since there have been no studies done to examine the effectiveness of anything used to date. To summarize, no single treatment modality has been reported to be proven effective for arresting this disease and there are no known treatments that prevent or control the disease itself.
Lymphangiomatosis & Gorham's Disease Alliance (LGD Alliance): http://www.lgdalliance.org email: This e-mail address is being protected from spambots. You need JavaScript enabled to view it
