S2420 Aseptic Systemic Abscess Syndrome: A Rare Syndromic Presentation of Crohn’s Disease

Abstract

Introduction: Aseptic systemic abscesses syndrome is a disorder of unknown etiology that involving polymorphonuclear neutrophil. Here we present an unusual syndromic manifestation of Crohn’s disease in a young patient presenting with multifocal aseptic abscesses, pyoderma gangrenosum, and hidradenitis suppurativa. Case description/methods: Case: A25 years old female presented with 2-days hx of RUQ pain, diarrhea and fever. She has hx notable for CD diagnosed in 2004. She had been treated in the past with adequate response to anti-TNF agents, she was unable to continue treatment for financial reasons. She also reported chronic diarrhea, associated with blood in the stool. She then developed worsening SOB. Workup was notable for elevated inflammatory markers (ESR 74/CRP 125.6). A CT abdomen noted hepatic and splenic hypodensities concerning for abscess(Figure 1). Started on multiple antibiotics regiments with no improvement. An extensive infectious disease workup was unremarkable and interventional radiology consulted for fluid aspiration which returned sterile frank pus. She continued to worsen, a CT chest, abdomen, and pelvis showed with interval enlargement of hepatic and splenic fluid collections(Figure 2). Gastroenterology consulted, EGD was unremarkable. Colonoscopy significant for a small segmental inflammation from 30-35 cm above the anal verge with biopsies consistent with Crohn’s colitis. Evaluation from rheumatology, hematology, and oncology was unremarkable. During prolonged hospital stay, she had inguinal and axillary abscesses with cellulitis along with associated skin ulceration involving her flank and breasts (Figure 3-5). Dermatology consulted, a diagnosis pyoderma gangrenosum and hidradenitis suppurativa was made. She continued to have chest pain and SOB, MRI chest revealed multiloculated fluid collection centered around the manubrium concerning for abscess with osteomyelitis(Figure 6,7). A mediastinal chest drain was placed for fluid drainage and cultures failed to grow any pathogen. Due to multiple autoimmune diseases and negative infectious workup, the manifestations were ascribed to their CD and a diagnosis of aseptic systemic abscess syndrome was made. Methylprednisone 40 mg daily initiated, then noted improvement in CRP with a resolution of her symptoms Discussion: Aseptic systemic abscess syndrome is a diagnosis of exclusion, delaying the treatment and expose the patients to extensive medical treatments and interventions. To the best of our knowledge, only a very few cases were reported.Figure 1.: Skin Biopsy and Cutaneous findings pre and post treatment.