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The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
Orpha Number: 54251
Corticosteroid-sensitive aseptic abscesses syndrome is a well-defined entity within the group of autoinflammatory disorders.
It is a rare disease with 49 cases documented so far. Prevalence is unknown.
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.
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.
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.
Source: GARD Last updated on 05-01-20
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