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

Disease 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: 8/13/2007

Symptoms Symptoms

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Elevated C-reactive protein level	Very frequent (present in 80%-99% of cases)
Fever	Very frequent (present in 80%-99% of cases)
Abnormality of the lymph nodes	Frequent (present in 30%-79% of cases)
Anemia	Frequent (present in 30%-79% of cases)
Elevated hepatic transaminases	Frequent (present in 30%-79% of cases)
Liver abscess	Frequent (present in 30%-79% of cases)
Neutrophilia	Frequent (present in 30%-79% of cases)
Weight loss	Frequent (present in 30%-79% of cases)
Abnormality of lung morphology	Occasional (present in 5%-29% of cases)
Abnormality of the pancreas	Occasional (present in 5%-29% of cases)
Abnormality of the testis	Occasional (present in 5%-29% of cases)
Brain abscess	Occasional (present in 5%-29% of cases)
Diarrhea	Occasional (present in 5%-29% of cases)
Myalgia	Occasional (present in 5%-29% of cases)
Abnormality of the kidney	Very rare (present in 1%-4% of cases)

Last updated: 10/1/2017

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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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