Case Report: Successful treatment of pyoderma gangrenosum-like granulomatous liver disease without skin lesions using a TNF-alpha inhibitor - Report - MDSpire
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Case Report: Successful treatment of pyoderma gangrenosum-like granulomatous liver disease without skin lesions using a TNF-alpha inhibitor
This case study presents a 73-year-old man with isolated hepatic and ocular involvement of pyoderma gangrenosum without skin lesions. The patient achieved complete clinical and biochemical remission following treatment with a TNF-alpha inhibitor.
Background
Pyoderma gangrenosum is a rare autoimmune condition primarily affecting the skin, but it can present with extracutaneous manifestations, including hepatic involvement. Recognizing these atypical presentations is crucial for timely diagnosis and appropriate management, as they can mimic other serious conditions and lead to misdiagnosis.
Data Highlights
Parameter
Value
Upper Limit Normal (ULN)
AST
128 U/L
34 U/L
ALT
116 U/L
59 U/L
CRP
147.5 mg/L
10 mg/L
Key Findings
The patient presented with fever, weight loss, and elevated liver enzymes.
Histological examination revealed sterile abscesses and granulomatous inflammation.
Extensive testing excluded common infectious and immunological causes.
Treatment with prednisone followed by infliximab led to symptom resolution.
This case highlights the systemic nature of pyoderma gangrenosum and the importance of considering it in differential diagnoses for granulomatous liver injury.
Clinical Implications
Clinicians should maintain a high index of suspicion for pyoderma gangrenosum in patients presenting with unexplained hepatic lesions, even in the absence of skin manifestations. Early recognition and treatment with TNF-alpha inhibitors may lead to significant clinical improvement.
Conclusion
This case underscores the need for awareness of extracutaneous manifestations of pyoderma gangrenosum and the potential for effective management with immunosuppressive therapies.
by Isabelle Panne, Matthias Matter, Jürg Vosbeck, Matthias von Rotz, Alexandra Steinemann, Marten Trendelenburg, Markus H. Heim, Peter Schirmacher, Andrea De Gottardi, Ansgar W. Lohse, Luigi M. Terracciano, Christine Bernsmeier
