Successful treatment of severe retroauricular pyoderma gangrenosum with upadacitinib in a patient with Crohn's disease and a history of cerebral venous sinus thrombosis.

Pyoderma gangrenosum (PG) is an immune-mediated skin condition that is commonly associated with inflammatory bowel diseases (IBD). Its diagnosis and management are often challenging due to the heterogeneity of the clinical presentation and the need for an individualized therapeutic approach. We present the case of a 19-year-old Caucasian male with Crohn's disease, who received therapy with a biological agent and developed severe PG in the retroauricular region following mastoidectomy for mastoiditis, which was complicated by cerebral venous sinus thrombosis. The diagnosis of PG was made with the help of the PARACELSUS-score based on lesion characteristics, histology, the patient's IBD history, and lack of response to antibiotics. Treatment with upadacitinib, a selective Janus kinase 1 inhibitor, was initiated after resolution of the previous thrombotic event. The patient responded rapidly to therapy, with a notable reduction of the local inflammatory activity and wound size, and ultimately complete healing. Anticoagulation therapy did not need to be continued during treatment. The patient has been on upadacitinib for 25 months to date without adverse events or recurrence of thrombosis. This case highlights upadacitinib as a promising and safe treatment option for PG, even in patients with Crohn's disease and a history of thrombotic events after careful benefit-risk assessment.