Corticosteroid-sensitive aseptic abscesses

Title

Other Names:

Aseptic abscesses syndrome; Aseptic systemic abscesses; Disseminated aseptic abscesses

Categories:

Skin Diseases

Summary Summary

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

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

Symptoms 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.

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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: 3/1/2020

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Learn More 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.

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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