Degos disease is a rare but deadly condition that develops mostly in patients with autoimmune disorders, including lupus erythematosus and antiphospholipid syndrome. It is also called malignant atrophic papulosis.
Dr. Cynthia M. Magro of Cornell University, New York, and colleagues provided a medical education article.
Small blood vessel damage is the characteristic feature of Degos disease. The skin, gastrointestinal tract, and central nervous system are the three main organ systems affected.
Magro reported that the inflammatory cytokine interferon-alpha is dysregulated in Degos patients, and the drug eculizumab (Soliris) may be helpful.
The Genetic and Rare Diseases Information Center (GARD) of the National Institutes of Health provides more information on Degos symptoms.
Skin lesions are the most obvious. GARD describes these as “porcelain-white macules” that develop primarily on the trunk, arms, and legs. These macules may be the only symptom for some patients, and are often the first symptom in patients who develop multi-organ damage.
Similar damage often occurs in the small intestines. Degos lesions can cause intestinal perforation, or tears, which must be treated as a medical emergency. Severe abdominal pain, along with chills, fever, nausea, and vomiting are the symptoms of perforation, according to MedlinePlus.
GARD lists central nervous system symptoms of Degos disease as partial paralysis, difficulty communicating, sensory disturbances and seizures.
The American Autoimmune Related Diseases Association (AARDA) reports that 20 percent of Degos patients develop central nervous system damage. Mental dysfunction, paresthesia (abnormal sensations), weakness of the limbs on one side, and other motor abnormalities are the symptoms listed on the AARDA web site.
Middle-aged people have the highest risk of Degos, according to GARD. Men are more likely to develop multi-organ damage, while women may be more likely to develop mild, benign forms of the disease.
Individuals with skin macules as the only symptoms have a good prognosis, while those with multi-organ damage often face life-threatening complications.
The Degos Disease Support Network offers online contact and support services for patients. Their website encourages input from all who are affected by the disease.
References:
Magro CM et al, “Degos disease: a C5b-9/interferon-alpha-mediated endotheliopathy syndrome”, Am J Clin Pathol 2011; 135: 599-610. http://www.ncbi.nlm.nih.gov/pubmed/21411783
Genetic and Rare Diseases Information Center. Degos Disease. Web. April 9, 2012.
http://rarediseases.info.nih.gov/GARD/QnASelected.aspx?diseaseID=6249
MedlinePlus. Gastrointestinal perforation. Web. April 9, 2012.
http://www.nlm.nih.gov/medlineplus/ency/article/000235.htm
American Autoimmune Related Diseases Association. Patient Information. Web. April 9, 2012.
http://www.aarda.org/patient_information.php
Degos Disease: The Degos Disease Support Network. Web. April 9, 2012.
http://www.degosdisease.com
Reviewed April 30, 2012
by Michele Blacksberg RN
Edited by Jody Smith