Corticosteroid-sensitive aseptic abscesses

Alternate names

Aseptic abscesses syndrome; Aseptic systemic abscesses; Disseminated aseptic abscesses

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.

Cause

The etiology is unknown: all searches for a pathogen, including those using PCR with universal and specific probes, remain negative.

Inheritance

A familial history of a granulomatous disorder is found in a few cases.

Signs and symptoms

• 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 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.

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

80%-99% of people have these symptoms

• Elevated C-reactive protein level
• Fever

30%-79% of people have these symptoms

• Abdominal pain(Pain in stomach)
• Abnormality of the lymph nodes(Abnormal lymph node histology)
• Anemia(Low number of red blood cells or hemoglobin)
• Elevated hepatic transaminase(High liver enzymes)
• Liver abscess
• Neutrophilia(Increased blood neutrophil counts)
• Weight loss

5%-29% of people have these symptoms

• Abnormal lung morphology(Abnormality of lung structure)
• Abnormal testis morphology(Abnormality of the testis)
• Abnormality of the pancreas
• Brain abscess
• Diarrhea(Watery stool)
• Myalgia(Muscle ache)

1%-4% of people have these symptoms

• Abnormality of the kidney(Abnormal kidney)

Diagnosis

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

Treatment

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

NIH genetic and rare disease info

• NIH info on Corticosteroid-sensitive aseptic abscesses

Corticosteroid-sensitive aseptic abscesses is a rare disease.

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