Background:
Pyoderma Gangrenosum (PG) is a rare auto-inflammatory disease, characterized by painful ulcerative skin-lesions often developing at sites of injury or surgery because of the typical pathergy phenomena. We describe an unusual case of PG after a caesarean section with excessive extra-cutaneous manifestation within internal organs.
Presentation of case:
A 21-year-old Dutch primigravida developed signs of sepsis after a caesarean section. Despite antibiotic treatment, fast clinical deterioration occurred. Exploration of the wound showed necrosis of the uterus and surrounding tissues. Due to the progression of necrosis, consecutive debridement procedures were executed resulting in a substantial abdominal wall defect. The progressive clinical course of the necrosis combined with absence of positive wound cultures and histology of prominent interstitial neutrophilic infiltration, led to the diagnosis 'Pyoderma Gangrenosum'. Treatment with high dose corticosteroids led to rapid regression of the disease. After several weeks, the abdominal wall defect was surgically corrected under systemic corticosteroid therapy.
Discussion:
This case of PG is unique due to the excessive extra-cutaneous presentation, which contributed to delayed diagnosis. Several surgical interventions in the active stage of disease resulted in expansion of PG and substantial morbidity for the patient.
Conclusion:
Post-operative PG can mimic infectious diseases, but treatment is substantially different. This case of extensive PG highlights the importance of timely recognition and treatment of the disease to reduce iatrogenic morbidity.