Progredient hepatosplenomegaly and skin lesions: a case report of extracutaneous pyoderma gangrenosum

Introduction: Progredient hepatosplenomegaly with focal lesions can be associated with various differential diagnoses. However, hepatosplenomegaly as a manifestation of extracutaneous pyoderma gangrenosum with sterile neutrophilic infiltration in the liver and spleen has not been rarely reported.

Objectives: This is a report of pyoderma gangrenosum-related hepatosplenomegaly with focal lesions in an adult.

Methods: This is a case report of a 28-year-old female hospitalized in January 2025 at University Hospital Mannheim. Diagnostics including imaging, biopsies, microbiological tests, endoscopy, and multidisciplinary discussions and clinical treatment were reported.

Results: The patient presented with progredient hepatosplenomegaly, newly identified liver and spleen lesions, and painful erythematous skin papulae on both legs. Laboratory results showed pronounced leukocytosis, significantly elevated CRP levels, and only minimally elevated procalcitonin. Serological testing ruled out infectious diseases including HIV, viral hepatitis, cytomegalovirus (CMV), Epstein-Barr virus (EBV), tuberculosis, and echinococcosis. Computer tomography imaging revealed subcapsular liver lesions. Biopsy of the lesions demonstrated histological findings of inflammation and granulocytic infiltration without cellular atypia. Dermatological punch biopsies of skin lesions revealed necrotic material, without pathogens in microbiological tests. Imaging characterized the subcutaneous skin lesions as nodular and without abscess suspicion. Further examinations ruled out vasculitis, inflammatory bowel disease, and malignancy. Recommendations from the autoimmune board led to lymph node excision and bone marrow puncture, excluding lymphoma and other hematological malignancies. Following multidisciplinary discussions, weight-adapted corticosteroid therapy and immunosuppressive treatment with ciclosporin were initiated. The patient responded well to this therapeutic approach with prompt laboratory and clinical improvements and was discharged to outpatient care with arrangements for regular follow-ups ([Fig. 1] [2] [3]).

Fig. 1  

Fig. 2  

Fig. 3  

Conclusion: For patients presenting with inflammatory skin lesions, extracutaneous neutrophilic diseases such as pyoderma gangrenosum should be considered as a potential differential diagnosis for hepatosplenomegaly. Effective diagnostics and management of such rare conditions require a multidisciplinary approach.

Publikationsverlauf

Artikel online veröffentlicht:
04. September 2025