Infliximab successfully treats pyoderma gangrenosum-like granulomatous liver disease without skin lesions
Background
Pyoderma gangrenosum (PG) is a rare, severe autoimmune condition primarily affecting the skin, often presenting as painful ulcers. However, extracutaneous manifestations, particularly hepatic involvement, are exceedingly rare and pose significant diagnostic challenges, especially when skin lesions are absent. The lack of typical cutaneous signs can delay diagnosis and appropriate treatment, as PG is often associated with underlying chronic inflammatory diseases. This case highlights the need to expand the clinical understanding of PG's systemic nature and improve recognition of its atypical presentations.
Study Design
A 73-year-old man presented with fever, 3 kg weight loss over 3 weeks, and elevated liver enzymes. Imaging revealed disseminated nodular liver lesions, and histology showed sterile abscesses and granulomatous inflammation. Ophthalmologic examination confirmed granulomatous uveitis. Extensive screening for infectious, rheumatological, malignant, and drug-induced causes yielded no confirmed diagnosis. Based on the distinctive histological pattern and exclusion of other conditions, pyoderma gangrenosum with hepatic and ocular involvement was assumed. Treatment began with prednisone, followed by the TNF-alpha inhibitor infliximab.
Results
The patient initially presented with significantly elevated liver parameters: AST at 128 U/L (upper limit norm [ULN]: 34 U/L) and ALT at 116 U/L (ULN: 59 U/L), alongside a markedly elevated CRP of 147.5 mg/L (ULN: 10 mg/L). Histology confirmed sterile abscesses and granulomatous inflammation in the liver. After initiating immunosuppressive treatment with prednisone and subsequently infliximab, the patient experienced a rapid and profound response. > This therapeutic strategy led to complete symptom resolution and full clinical and biochemical remission, normalizing liver enzymes and inflammatory markers. The successful outcome underscores the role of TNF-alpha inhibition in managing severe, atypical manifestations of pyoderma gangrenosum.
Key Findings
- A 73-year-old man presented with isolated granulomatous liver disease and uveitis, lacking typical pyoderma gangrenosum skin lesions.
- Initial presentation included elevated AST (128 U/L), ALT (116 U/L), and CRP (147.5 mg/L).
- Histology revealed sterile abscesses and granulomatous inflammation in the liver.
- Treatment with prednisone followed by infliximab led to complete symptom resolution and biochemical remission.
Why It Matters
This case significantly expands the clinical spectrum of pyoderma gangrenosum, reinforcing its systemic nature and the critical importance of considering it even in the absence of characteristic skin lesions. Clinicians should be aware that PG can present solely with extracutaneous manifestations, such as granulomatous liver disease and uveitis, necessitating a high index of suspicion for early diagnosis. The successful use of infliximab highlights TNF-alpha inhibition as an effective therapeutic strategy for these challenging, atypical presentations. This finding suggests that a TNF-alpha inhibitor could be a crucial component of a treatment protocol for severe, refractory, or atypical PG, potentially preventing prolonged diagnostic odysseys and organ damage.