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A 21-year-old woman Lakshmi (Name changed) from Khammam was suffering from a unique autoimmune disease, Aseptic abscess syndrome with pyoderma gangrenosum which has gone undiagnosed for a long time. She was given intensive antibiotic therapy but no response and advised removal of spleen in the hospitals where she visited earlier. She was then referred to Kamineni Hospitals, Hyderabad.
What Is Aseptic Abscess Syndrome?
Aseptic Abscess syndrome is an autoinflammatory disease with fever, abdominal pain, and abscesses usually involving the spleen, liver; and associated with Inflammatory Bowel Disease in the majority of the cases. Cutaneous lesions are present only in 20 per cent of the cases and are mainly cutaneous abscesses. It is often a relapsing condition, and the CT scan may be an appropriate diagnostic tool in the setting of recurrent episodes of fever and pain in the abdomen. Lab tests like ESR, CRP are often elevated with elevated white blood count in the basic blood pictures.
What Causes Aseptic Abscess Syndrome?
An aseptic abscess syndrome is either associated with another disease such as Crohn's disease or relapsing polychondritis or is idiopathic. This condition tends to recur frequently. The aseptic abscesses syndrome shares some characteristics with other conditions such as inflammatory bowel disease or neutrophilic dermatoses, suggesting a genetic susceptibility and a relationship with auto-inflammatory conditions.
How Was Aseptic Abscess Syndrome Treated In This Case?
Doctors at Kamineni Hospitals had successfully treated this rare disease, Aseptic abscess syndrome with pyoderma gangrenosum. She was not given a solid diagnosis or treatment elsewhere for high fever, nodules and multiple ulcers over both hands and legs of sudden onset. On ultrasonography, multiple abscesses in the spleen were noted.
"We had examined the patient's condition with her complete medical history and did an ultrasound abdomen, skin biopsy, and basic blood tests. The patient had been diagnosed with a rare condition that affects the skin and internal organs severely called aseptic abscess syndrome with pyoderma gangrenosum. We have given her steroid injections under strict monitoring and liquidated her with immunomodulator tablets. Within 2 months, Abscesses in the spleen resolved but the cutaneous ulcers were resistant to tablets. Hence, we had started biological injections (newer therapies) sequentially under strict supervision with follow-ups, which helped with the overactive immune system that was acting against her and patient got back to her normal health status" said Dr Ramadevi B, Consultant Dermatologist, Kamineni Hospitals, LB Nagar.
"They were about to remove her spleen, but we are extremely happy to salvage it, the puzzle as to why this young girl deteriorated all of a sudden was solved. We are beyond happy to develop an abundance of trust with this patient and her family" Dr Ramadevi B, Consultant Dermatologist, Kamineni Hospitals added further.
With inputs from Kamineni Hospitals, Hyderabad
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