Signs and symptoms of Degos disease can vary greatly from person to person. Skin symptoms include porcelain-white macules that tend to develop on the trunk, arms, and legs. For some people, this is their only symptom. For others, Degos disease affects multiple body organs. Skin macules tend to be the earliest symptom in multisystem disease. Small bowel involvement is very common, and Degos disease can cause intestinal perforation (tear). Intestinal perforation is a medical emergency which requires prompt treatment. Unfortunately intestinal disease tends to recur in these individuals.

Degos disease can also affect the nervous system, in particular the cerebral and peripheral nerves. This may result in a variety of symptoms, such as partial paralysis, aphasia (difficulty communicating), cranial neuropathies (which affect nerves that are connected with the brain and control sight, eye movement, hearing, and taste), sensory disturbances, and seizures.

Currently, there is not a targeted therapy for Degos disease that has been proven effective. Treatment of Degos disease has been attempted with antithrombotic agents, such as aspirin and dipyridamole. These treatments were reported to be effective in some patients. Other treatments that have been tried, but have shown inconsistent results, include anticoagulants and fibrinolytic agents (drugs to help break-up and dissolve clots), ticlopidine, pentoxifylline, prostaglandin E1, and interferon alpha-2a. Treatment with intravenous immunoglobulin has also been tried, but produced conflicting results. Infliximab was reported to be ineffective in one case. Immunosuppressives such as corticosteroids may worsen Degos disease.

For further information on your treatment options, we encourage you to discuss your questions and this information with your healthcare provider.