DEGOS DISEASE

ALTERNATE NAMES

Degos-Kohlmeier Disease; Degos Syndrome; Kohlmeier-Degos Disease;

Malignant Atrophic Papulosis; Kohlmeier disease

DESCRIPTION

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.

DIAGNOSTIC

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.)

ONSET AND PROGRESSION

Systemic Degos disease is frequently fatal within 2-3 years from the onset of systemic involvement.

TREATMENT

Treatment options are limited, consisting mainly of antiplatelet drugs, anticoagulants or immunosuppressants . The effect of treatment is limited to case reports.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation: Degos disease is a disease of the blood vessels. Since these individuals are initially seen by a dermatologist, the dermatological exam may assist with determining onset. This condition should be evaluated according to the ability of the individual to adjust to the reduced oxygen carrying capacity of the blood. Chronicity is indicated by persistence of the condition for at least 3 months. Clinical examination including a physical description of findings. Laboratory tests measuring blood composition. MRI/CT scans may also be needed to evaluate this condition.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

14.03

Differentiate from benign form (Degos acanthoma) that only affects skin.

Medical Equals