Behçet’s disease complicated by multiple aseptic abscesses of the liver and spleen

Abstract

Aseptic abscesses are an emergent entity and have been described in inflammatory bowel disease, especially in Crohn's disease, and in other diseases. However, aseptic abscesses associated with Behçet's disease are extremely rare. We report a Japanese male diagnosed with an incomplete type of Behçet's disease who developed multiple aseptic abscesses of the spleen and liver. In 2002, the spleen abscesses were accompanied by paroxysmal oral aphthous ulcers and erythema nodosum. As the patient's response to antibiotic treatment was inadequate, a splenectomy was performed. Severe inflammatory cell infiltration, largely of polymorphonuclear neutrophils, was observed without evidence of bacterial or fungal growth. Although the patient had no history of ocular symptoms or genital ulcers, a diagnosis of incomplete Behçet's disease was made according to the Japanese diagnostic criteria because of the presence of paroxysmal arthritis and epididymitis since 2002. In 2005, multiple liver abscesses developed with right hypochondrial pain and seemed to be attributed to Behçet's disease because the abscesses yielded negative results during a microbiologic investigation and failed to go into remission under antibiotic therapy. Oral prednisone (15 mg/d) was started in May 2006, and the abscesses dramatically disappeared 4 wk after treatment. Although the patient had a relapse of the liver abscesses in association with the tapering of prednisone, the augmentation of prednisone dosage yielded a response. The abscesses of the liver and spleen were strongly suggested to be attributed to Behçet's disease. Clinician should be aware of the existence of aseptic abscesses as uncommon manifestations of Behçet's disease.

Multiple Hepatic Micro-Hypodensities as a Presenting Sign in Systemic Lupus Erythematosus- A Case Report

“…Aseptic liver abscesses have been described in inflammatory bowel disease, particularly Crohn's, but are rare in systemic inflammatory conditions [9]. Maeshima et al reported a case of aseptic abscesses in a patient with Behcet's disease that improved with corticosteroid therapy [10,11]. The diagnosis of Behçet's was considered based on her reported painful oral and vaginal lesions but similar lesions in SLE have also been reported [12].…”

Behçet's disease with multiple splenic abscesses in a child

“…Among adults, AAs accompanied by BD are also rarely reported. A case was recently reported in which multiple abscesses were observed in the liver and spleen 12. The steroid dosage was increased when the condition recurred after initial steroid tapering, and the AAs eventually improved.…”

“…A case was recently reported in which multiple abscesses were observed in the liver and spleen. 12 The steroid dosage was increased when the condition recurred after initial steroid tapering, and the AAs eventually improved. However, there have been no reports of AAs associated with BD in children.…”

“… 7 In systemic diseases in both children and adults, AAs have been reported, mostly in association with IBD and sometimes with Sweet's syndrome or pyoderma gangrenosum. 7 8 9 10 11 AAs associated with BD are rare in adults 12 and has not been reported in children.…”

Persistent Fever and Abdominal Pain in a Young Woman With Budd-Chiari Syndrome

“…Few cases of aseptic abscesses in BD successfully treated with corticosteroid therapy have been described. 3 Although this condition is an uncommon manifestation of BD, it is important for physicians to be aware of this possibility to avoid any delay in diagnosis and unnecessary medical and surgical measures.…”