Behçet's Disease with Hepatic Involvement
The combination of oral-genital ulcers and multiple liver abscesses strongly suggests Behçet's disease with aseptic hepatic abscesses, requiring immediate corticosteroid therapy (prednisone 30-60 mg or 1 mg/kg daily) after excluding infectious causes. 1
Diagnostic Approach
Immediate Workup Required
The triad of oral ulcers, genital ulcers, and multiple liver abscesses is pathognomonic for Behçet's disease with visceral involvement, though this presentation is extremely rare. 1 Before initiating immunosuppressive therapy, you must exclude infectious etiologies:
Critical laboratory tests:
- Full blood count to detect leukemia, neutropenia, or anemia 2, 3
- HIV antibody and syphilis serology (both can cause oral-genital ulcers) 2, 3
- Fasting blood glucose (hyperglycemia predisposes to invasive fungal infections) 2, 3
- 1-3-β-D-glucan and galactomannan levels if fever present (to exclude invasive fungal infection) 3
- Blood cultures and liver abscess aspiration for bacterial, fungal, and amebic cultures 1, 4
Imaging confirmation:
- CT scan already obtained showing multiple liver abscesses 1
- Consider additional imaging to assess for splenic involvement 1
Key Diagnostic Features of Behçet's Disease
Look for these additional manifestations to confirm the diagnosis:
- Recurrent oral aphthous ulcers (required criterion) 1
- Genital ulcers 1
- Ocular symptoms (uveitis, though may be absent in incomplete Behçet's) 1
- Skin lesions (erythema nodosum, papulopustular lesions) 1
- Arthritis 1
- Epididymitis 1
The patient described had incomplete Behçet's disease with paroxysmal oral aphthous ulcers, erythema nodosum, arthritis, and epididymitis, which developed aseptic abscesses of both spleen and liver. 1
Treatment Algorithm
Step 1: Rule Out Infectious Causes
If cultures are negative and antibiotics fail after 7-10 days, strongly suspect aseptic abscesses from Behçet's disease. 1 The key distinguishing feature is that aseptic abscesses show severe polymorphonuclear neutrophil infiltration without bacterial or fungal growth and fail to respond to antimicrobial therapy. 1
Step 2: Initiate Corticosteroid Therapy
Start oral prednisone 30-60 mg daily (or 1 mg/kg) immediately once infectious causes are excluded. 5, 3 In the reported case, prednisone 15 mg daily caused dramatic disappearance of liver abscesses within 4 weeks. 1
Critical pitfall: Do not taper corticosteroids prematurely before disease control is established, as this leads to relapse. 5, 3 The patient in the case report relapsed when prednisone was tapered, requiring dose augmentation. 1
Step 3: Add Colchicine as First-Line Systemic Therapy
Colchicine should be started concurrently, especially given the presence of both oral and genital ulcers. 5, 3 Colchicine is particularly effective for erythema nodosum and genital ulcers in Behçet's disease. 5, 3
Step 4: Concurrent Symptomatic Management
While awaiting response to systemic therapy, provide aggressive topical management:
For oral ulcers:
- Clobetasol propionate 0.05% gel for localized lesions 3
- Betamethasone sodium phosphate 0.5 mg in 10 mL water as rinse-and-spit four times daily for widespread ulcers 5, 3
- Benzydamine hydrochloride rinse every 3 hours before eating 5, 3
- Viscous lidocaine 2% before meals for pain control 5, 3
For genital ulcers:
Step 5: Escalation for Refractory Cases
If abscesses persist or recur despite adequate corticosteroid therapy:
- Azathioprine as steroid-sparing agent 5, 3
- Interferon-alpha 5, 3
- TNF-alpha inhibitors (infliximab, adalimumab) 5, 3
- Apremilast for severe cases 5, 3
Critical Pitfalls to Avoid
Never rely solely on antibiotics for multiple liver abscesses with oral-genital ulcers without considering Behçet's disease. 3 The failure to respond to antimicrobial therapy is a key diagnostic clue. 1
Do not perform splenectomy or hepatic drainage procedures for aseptic abscesses once Behçet's is diagnosed. 1 The patient in the case report underwent unnecessary splenectomy before the diagnosis was established. 1 These abscesses respond dramatically to corticosteroids alone. 1
Avoid inadequate diagnostic workup that misses systemic causes. 2, 3 Overlooking Behçet's disease leads to delayed diagnosis, inappropriate surgical interventions, and prolonged morbidity. 2, 3
Do not taper steroids too quickly. 5, 3 Maintain adequate dosing until complete resolution of abscesses is confirmed on repeat imaging, then taper slowly over weeks to months. 1