What is Ledderhose Disease?
Ledderhose disease (LD) is a rare form of cancer which occurs mainly in children. It is caused by a virus called Epstein Barr Virus (EBV). EBV causes lymphoma, but it can also cause other types of cancers such as leukemia, Hodgkin’s Lymphoma, non-Hodgkin’s Lymphoma and many others. LD affects mostly the bone marrow and blood cells.
The main symptoms are fever, fatigue, loss of appetite, weight loss and skin rashes. If left untreated the patient may die within one year. There is no cure for LD and treatment consists of chemotherapy and radiation therapy. Treatment with radiation therapy usually involves removal of affected organs such as the liver or lungs. Other treatments include chemotherapy drugs used to treat patients with cancer.
However these drugs have side effects which make them less effective in treating LD cases especially when combined with radiation therapy.
In some cases, the cancer spreads outside the body and into nearby organs like the bones or brain. These cases are very rare and they require further investigation. Currently there is no known vaccine against LD.
How common is Ledderhose Disease?
According to the World Health Organization (WHO), LD accounts for only 0.1% of all childhood cancers worldwide. Most of these cases occur in Africa where it makes up between 1-5% of all childhood cancers.
LD is more common in boys than girls, although no exact reason is known.
However, children who have a family history of cancer are prone to getting the disease. The risk of getting it is also increased if the person was infected with the virus that causes LD.
Children infected with the human immunodeficiency virus (HIV) are also at high risk of getting it.
How is Ledderhose Disease caused?
LD is caused by the human immunodeficiency virus (HIV). This virus breaks down a person’s immunity system, making it very easy for other diseases and viruses to attack the body. However, 90% of people who are infected with HIV will not experience any symptoms at all. It is only when the immune system becomes severely damaged that various types of cancerous diseases start attacking various organs in the body. This is the case with LD.
The Epstein Barr virus (EBV) causes the Ledderhose disease. This is a common virus which infects more than 90% of the population at some point in their life. It is spread through bodily fluids like saliva, blood and other secretions. The virus lies dormant and becomes activated when your immune system is weakened. This is why it mostly affects people who have a compromised immune system such as people living with HIV or AIDS.
Sources & references used in this article:
Ledderhose disease: an unusual presentation by DF de Souza, L Micaelo, T Cuzzi… – The Journal of clinical …, 2010 – ncbi.nlm.nih.gov
Ledderhose disease: clinical, radiological (ultrasound and MRI), and anatomopathological findings by Y Omor, B Dhaene, S Grijseels, S Alard – Case reports in Orthopedics, 2015 – hindawi.com
Collagenase Clostridium histolyticum injection for plantar fibromatosis (Ledderhose disease) by ZS Hammoudeh – Plastic and reconstructive surgery, 2014 – cdn.journals.lww.com
Association of Morbus Ledderhose with Dupuytren’s contracture by KG Gudmundsson, T Jónsson… – Foot & ankle …, 2013 – journals.sagepub.com