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.