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Disease Profile

Drug reaction with eosinophilia and systemic symptoms

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Drug rash with eosinophilia and systemic symptoms; DRESS syndrome; Drug reaction eosinophilic systemic syndrome

Summary

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
orphanet

Orpha Number: 139402

Definition
A rare hypersensitivity reaction characterized by a generalized skin rash, fever, eosinophilia, lymphocytosis and visceral involvement (hepatitis, nephritis, pneumonitis, pericarditis and myocarditis) and, in some patients, reactivation of human herpes virus 6.

Epidemiology
Prevalence is unknown.

Etiology
Onset usually occurs 2-6 weeks after administration of the causal medication. DRESS syndrome is most frequently associated with anticonvulsants and sulfonamides but other medications (allopurinol, cyclosporine, azathioprine, gold salts and antiviral agents) have also been implicated. The manifestations may persist for several weeks after withdrawal of the causative medication.

Visit the Orphanet disease page for more resources.

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Angioedema
0100665
Cardiac arrest
Heart stops beating
0001695
Encephalitis
Brain inflammation
0002383
Erythema
0010783
Erythroderma
0001019
Fever
0001945
Immunologic hypersensitivity
0100326
Lymphadenopathy
Swollen lymph nodes
0002716
Macule
Flat, discolored area of skin
0012733
Skin rash
0000988
Thyroiditis
Thyroid gland inflammation
0100646
Weight loss
0001824
30%-79% of people have these symptoms
Elevated hepatic transaminase
High liver enzymes
0002910
Interstitial pneumonitis
0006515
Lymphocytosis
High lymphocyte count
0100827
Pulmonary infiltrates
Lung infiltrates
0002113
5%-29% of people have these symptoms
Acute hepatic failure
Acute liver failure
0006554
Cough
Coughing
0012735
Dyspnea
Trouble breathing
0002094
Enanthema
0030249
Eosinophilia
High blood eosinophil count
0001880
Hepatitis
Liver inflammation
0012115
Myocarditis
Inflammation of heart muscle
0012819
Nephrotic syndrome
0000100
Peripheral neuropathy
0009830
Pustule
Pimple
0200039
Renal insufficiency
Renal failure
Renal failure in adulthood

[ more ]

0000083
Tubulointerstitial nephritis
0001970

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Social Networking Websites

    • Visit the following Facebook groups related to Drug reaction with eosinophilia and systemic symptoms:
      DRESS Syndrome

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • The American Academy of Allergy Asthma and Immunology offers information on Drug reaction with eosinophilia and systemic symptoms
      • DermNet New Zealand is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
      • The The Cleveland Clinic Web site has an information page on Drug reaction with eosinophilia and systemic symptoms. Click on the Cleveland Clinic link to view this page.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.