DEGOS DISEASE - SYSTEMIC
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ALTERNATE NAMES
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Degos-Kohlmeier Disease; Degos Syndrome; Kohlmeier-Degos Disease; Malignant Atrophic
Papulosis; MAP; Kohlmeier disease; Papulosis Atrophican Maligna; Degos’s Malignant
Atrophic Papulosis; Atrophic Papulosis, malignant
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DESCRIPTION
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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. Immune system dysregulation may be a key driver in Degos disease
development.
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). At Stage 2, the disease is usually fatal within 2 or 3 years.
The disorder usually occurs in young adults, mostly affecting men. The cause of this
disease is unknown.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: The diagnosis of systemic Degos disease is made by:
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Identification of characteristic findings (e.g. skin lesions);
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Microscopic examination of affected skin tissue that reveals distinctive changes to
the tissue.
Physical findings: Degos disease is characterized by:
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Erythematous pink or red papules on the skin (skin lesions);
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Lesions in the gastrointestinal tract when there is gastric involvement; and
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Lesions with central porcelain-white atrophy and surrounding teleangiectatic rim.
ICD-9: 447.8
ICD-10: I77.89
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TREATMENT
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Treatment options are limited, consisting mainly of antiplatelet drugs, anticoagulants
or immunosuppressants. The effect of treatment is limited to case reports.
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PROGRESSION
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Systemic Degos disease is frequently fatal within 2-3 years from the onset of systemic
involvement.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Dermatology consultation that evaluates the ability of the individual to adjust to
the reduced oxygen carrying capacity of blood;
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Laboratory tests measuring blood composition; and
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Suggested Listings for Evaluation:
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DETERMINATION |
LISTING
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REMARKS
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Meets
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Equals
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14.03
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Degos disease is not a vasculitis, it is an occlusive arteriopathy. Therefore, it
can not meet listing 14.03. The relative effectiveness of eculizumab, a hematologic
medication which blocks complement component 5 (C5) suggests it may be a hematologic/endothelial/clotting
disease. Along with the skin, organ and body systems most commonly affected include
gastrointestinal, neurologic, cardiac, and pulmonary. Constitutional symptoms or signs
often include fatigue, persistent fever, and involuntary weight loss.
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114.03 |
The relative effectiveness of eculizumab, a hematologic medication which blocks complement
component 5 (C5) suggests it may be a hematologic/endothelial/clotting disease. Along
with the skin, organ and body systems most commonly affected include gastrointestinal,
neurologic, cardiac, and pulmonary. Constitutional symptoms or signs often include
fatigue, persistent fever, and involuntary weight loss.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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