This disease is a result of a lymphatic malformation or abnormal development of the lymphatic system, also known as a lymphangiomia. The underlying cause of this rare disease is unknown in many of the cases. However, various studies have been conducted in regards to the pathology of this disease. The pathologic process involves the collection of lymphatic cisterns within the deep subcutaneous layer of the skin. These are separated from the fundamental network of lymph vessels. Communication between the superficial lymph vessels is achieved through dilated and vertical lymph channels. The malformation arises from when the primitive lymph sac is unable to connect with the rest of the lymphatic system during embryonic development. This disruption leads to the production of a thick coat of muscle fibers that cause rhythmic contractions which increase the intramural pressure. The added pressure causes the dilated channels to extend from the deep walls of the cisterns outward to the skin. Findings from a study performed in 1976 by Whimster, suggested that the vesicles noted in lymphangioma are actually outpouchings of the dilated projected vessels. This idea was furthermore supported by radiographic studies that displayed large multiobulated cisterns that lay deep into the dermis and beyond clinical lesions. These studies concluded that lymphangiomas display no evidence of communication with the rest of the normal lymphatics. Furthermore emphasizing the idea that the cause of this disease is a failure of primitive lymph sacs to connect with the lymphatic system.
Lymphohemangioma
Lymphangioma
Chorangioma
Chorioangioma
Tufted angioma
Spider angioma
Hemangioma
Angiokeratoma