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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Degos disease


Other Names for this Disease

  • Atrophic papulosis, malignant
  • Degos syndrome
  • Degos's malignant atrophic papulosis
  • Kohlmeier-Degos disease
  • Malignant atrophic papulosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Symptoms

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What are the signs and symptoms of Degos disease?

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).[1] Intestinal perforation is a medical emergency which requires prompt treatment. Click here to learn more about the signs and symptoms of intestinal perforation.  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.[1]
Last updated: 6/6/2011

The Human Phenotype Ontology provides the following list of signs and symptoms for Degos disease. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Aplasia/Hypoplasia of the skin 90%
Teleangiectasia of the skin 90%
Abdominal pain 50%
Gastrointestinal hemorrhage 50%
Gastrointestinal infarctions 50%
Malabsorption 50%
Muscle weakness 50%
Nausea and vomiting 50%
Weight loss 50%
Abnormality of the myocardium 7.5%
Abnormality of the pericardium 7.5%
Abnormality of the pleura 7.5%
Abnormality of the urinary system 7.5%
Amaurosis fugax 7.5%
Arterial thrombosis 7.5%
Cataract 7.5%
Cerebral ischemia 7.5%
Chest pain 7.5%
Coronary artery disease 7.5%
Cranial nerve paralysis 7.5%
Diplopia 7.5%
Intestinal fistula 7.5%
Migraine 7.5%
Optic atrophy 7.5%
Paresthesia 7.5%
Peritonitis 7.5%
Ptosis 7.5%
Respiratory insufficiency 7.5%
Seizures 7.5%
Vasculitis 7.5%
Vertigo 7.5%
Abnormality of the conjunctiva -
Autosomal dominant inheritance -
Constrictive pericarditis -
Gastrointestinal hemorrhage -
Gastrointestinal infarctions -
Papule -
Pleural effusion -
Stroke -

Last updated: 11/3/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Mark LA, Mirowski GW. Oral Disease and Oral-Cutaneous Manifestations of Gastrointestinal and Liver Disease. In: Feldman eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed. Philadelphia, PA: Saunders; 2010;
  2. Wilson J . Benign cutaneous Degos disease in a 16-year-old girl. Pediatr Dermatol. 01-JAN-2007;


Other Names for this Disease
  • Atrophic papulosis, malignant
  • Degos syndrome
  • Degos's malignant atrophic papulosis
  • Kohlmeier-Degos disease
  • Malignant atrophic papulosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.