Case Report: Successful treatment of pyoderma gangrenosum-like granulomatous liver disease without skin lesions using a TNF-alpha inhibitor.

Pyoderma gangrenosum is a rare autoimmune condition that is difficult to diagnose and primarily affects the skin. Extracutaneous manifestations, including hepatic involvement, occur only rarely. Here, we report a case of extracutaneous manifestation of pyoderma gangrenosum without adjunct skin lesions, emphasizing the diagnostic challenges, immunological considerations, and therapeutic implications. This case describes a 73-year-old man presenting with isolated hepatic and ocular involvement of pyoderma gangrenosum, without skin lesions and without an underlying chronic inflammatory disease. This case aims to expand the clinical understanding of the spectrum of pyoderma gangrenosum, reinforce its systemic nature, and contribute to improved recognition of extracutaneous disease involvement even without primary skin manifestation. The patient exhibited fever, weight loss of 3 kg within 3 weeks, elevated hepatocellular and cholestatic liver parameters (AST: 128 U/L, upper limit norm [ULN]: 34 U/L; ALT: 116 U/L, ULN: 59 U/L), and elevated CRP (147.5 mg/L, ULN: 10 mg/L). Imaging revealed disseminated nodular liver lesions. Histology demonstrated sterile abscesses and granulomatous inflammation. Ophthalmologic examination revealed conjunctival injections and granulomatous uveitis. No other organs were involved. Extensive testing excluded infectious or other immunological causes. After comprehensive screening for infectious, rheumatological, malignant, or hematological diseases, systemic inflammatory syndromes, and drug-induced liver injury, none of the suspected common diagnoses could be confirmed. In relation to the distinctive histological pattern resembling a few published case reports, we assumed pyoderma gangrenosum with hepatic and ocular involvement without skin lesions. We initiated immunosuppressive treatment with prednisone, followed by infliximab. This strategy successfully led to symptom resolution and complete clinical and biochemical remission. This case highlights the diagnostic challenges of granulomatous liver injury and pyoderma gangrenosum at unusual sites. Pyoderma gangrenosum is a rare condition, particularly when presenting with isolated hepatic and ocular involvement, and may be considered a differential diagnosis underlying granulomatous hepatic injury. Using a TNF-alpha inhibitor, we successfully achieved a complete remission.