Degos disease is a rare non-inflammatory disease of the blood vessels that is characterized by a narrowing and blockage in small and medium sized arteries, leading to ischemia and tissue infarction in the involved organ systems. Skin lesions are the characteristic feature of Degos disease. Blood vessels that supply blood to the skin, gastrointestinal tract, central nervous system, and organs such as the eyes, kidneys, heart, and liver can also be involved. The disease occurs in two stages. Stage 1 which involves skin lesions that may last for weeks to years and Stage 2 which involves lesions of the intestines and other organs. In the first stage, individuals will notice red lesions that leave scars with white centers. These lesions can result in bowel ischemia, chronic skin lesions, ocular lesions, strokes, spinal lesions, mononeuritis multiplex (inflammation of several separate nerves), epilepsy, headaches, or cognitive disorders. In the second stage, the more lethal multi-organ variant form, individuals begin to complain of abdominal pain, diarrhea, and/or weight loss. The intestinal lesions are known to break through the wall of the bowels (perforation), thus leading to a potentially life threatening complication (sepsis). The disorder usually occurs in young adults, mostly affecting men. The cause of this disease is unknown.
TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
CT scan, MRI, blood tests, and clinical evaluation. Biopsy and surgical pathology specimens (if found in the MER) may be helpful in diagnosis.
ICD-9: 447.8 (Other specified disorders of arteries and arterioles.)