Spontaneous skin necrosis revealing protein S deficiency in Crohn's disease

Area of Science:

• Gastroenterology
• Dermatology
• Hematology

Background:

• Inflammatory bowel diseases (IBD) are linked to hypercoagulability, increasing thrombosis risk.
• Protein S deficiency is a known, albeit uncommon, cause of hypercoagulability in IBD patients.
• Spontaneous skin necrosis is a rare manifestation, particularly in adults with Crohn's disease.

Observation:

• A 35-year-old woman with active Crohn's disease developed extensive spontaneous skin necrosis.
• The patient was diagnosed with protein S deficiency, identified as the cause of the necrosis.
• The condition presented as a rare cutaneous manifestation in an adult IBD patient.

Findings:

• Successful treatment involved vascular filling, anticoagulation, antibiotics, and high-dose corticosteroids.
• The patient experienced a favorable outcome following the comprehensive treatment regimen.
• Protein S deficiency was confirmed as the underlying cause of the extensive skin necrosis.

Implications:

• This case underscores the importance of recognizing skin necrosis as a potential complication of IBD.
• Early diagnosis and management of protein S deficiency are crucial in IBD patients presenting with skin necrosis.
• Considering cutaneous manifestations like skin necrosis can lead to timely diagnosis and improved patient outcomes in IBD.