Degos disease is an extremely rare disease. It is also called Köhlmeier-Degos disease. It is a fatal disease and is life-threatening. In this disease, the small and medium-sized arteries in the body get blocked, resulting in restricted blood flow to affected parts. It is a blood vessel disorder, and its signs and symptoms vary from one individual to another. There is no effective treatment for Degos disease as of now, and the treatment is often targeted towards the particular symptoms in each patient. There is very little knowledge on how this disease actually works. Following are the treatment that have been tried and tested to treat this disease:
There have been attempts to treat it using antithrombotic agents. These were found to be effective in a few individuals.
Immunosuppressive agents like corticosteroids have been tested, but it was found that they worsened the disease. Thus, this treatment plan was unsuccessful.
Fibrinolytic agents, anticoagulants, and prostaglandin E1 have been tried as treatments, but they failed to produce the desired results.Doctors are uncertain regarding which medicine should be used for the treatment.
Intravenous immunoglobulin treatment is another hit and trial method that might help in the treatment of this disease.Infliximab was found to be effective in some cases.
Experimental treatment using eculizumab has benefitted some individuals. But this is very expensive and needs to be infused after every fourteen days.
According to reports, the use of Treprostinil helped in clearing the gastrointestinal tract and CNS. In some patients, Degos disease affects the gastrointestinal tract. Thus, regular monitoring can help detect abnormalities in the intestines. Sometimes surgical procedures may be necessary.