National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Corticosteroid-sensitive aseptic abscesses


Other Names:
Aseptic abscesses syndrome; Aseptic systemic abscesses; Disseminated aseptic abscesses
Categories:
The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
orphanet

Orpha Number: 54251

Definition
Corticosteroid-sensitive aseptic abscesses syndrome is a well-defined entity within the group of autoinflammatory disorders.

Epidemiology
It is a rare disease with 49 cases documented so far. Prevalence is unknown.

Clinical description
It affects mainly young adults and is characterized by recurrent attacks of fever and deep abscess-like collections, most frequently localized in the abdomen. Blood markers of inflammation and polymorphonuclear neutrophil levels are elevated. Aseptic abscesses may be either isolated or associated with an underlying condition such as relapsing polychondritis (see this term) or inflammatory bowel disease. The abscesses usually precede the diagnosis of inflammatory bowel disease, possibly by several years. A neutrophilic dermatosis, like pyoderma gangrenosum, may also be observed.

Etiology
The etiology is unknown: all searches for a pathogen, including those using PCR with universal and specific probes, remain negative. A familial history of a granulomatous disorder is found in a few cases.

Diagnostic methods
On pathologic examination, aseptic abscesses consist of a core of altered polymorphonuclear leukocytes surrounded by palisading histiocytes and sometimes giant cells.

Management and treatment
Antibiotics fail to cure the patients but dramatic improvements are seen with corticosteroids and immunosuppressive drugs.

Visit the Orphanet disease page for more resources.
Last updated: 8/1/2007

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.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Elevated C-reactive protein level 0011227
Fever 0001945
30%-79% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Abnormality of the lymph nodes
Abnormal lymph node histology
0002733
Anemia
Low number of red blood cells or hemoglobin
0001903
Elevated hepatic transaminase
High liver enzymes
0002910
Liver abscess 0100523
Neutrophilia
Increased blood neutrophil counts
0011897
Weight loss 0001824
5%-29% of people have these symptoms
Abnormal lung morphology
Abnormality of lung morphology
Abnormality of the lungs
Abnormally shaped lung
Unusal lung shape
[ more ] 		0002088
Abnormal testis morphology
Abnormality of the testis
0000035
Abnormality of the pancreas 0001732
Brain abscess 0030049
Diarrhea
Watery stool
0002014
Myalgia
Muscle ache
Muscle pain
[ more ] 		0003326
1%-4% of people have these symptoms
Abnormality of the kidney
Abnormal kidney
0000077
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Last updated: 7/1/2020

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.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Corticosteroid-sensitive aseptic abscesses. Click on the link to view a sample search on this topic.

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