Title : Bullous pyoderma gangrenosum associated with anti-phospholipid syndrome: Case report and literature review
Abstract:
We report the rare case of a 49-year-old Omani woman who is known to have primary anti-phospholipid syndrome (APS), glucose-6-phosphate dehydrogenase deficiency (G6PD), and Iron deficiency anemia (IDA). She was found to develop bulla that progressed to ulcerations after cannulation. The chronicity and the abscess formation that increased after surgical intervention led to the postulation of a pathergy phenomenon. A high suspicion of pyoderma gangrenosum (PG) was considered. Fortunately, the rapid progression of the disease was slowed down with corticosteroids, cyclosporin, and biologic agents.
APS is a prothrombotic condition defined by the presence of anti-phospholipid antibodies and manifests clinically as recurrent morbidity during pregnancy and/or thromboembolic complications. [1] The cutaneous manifestations of APS vary from livedo reticularis to cutaneous necrosis. Ulcers resembling PG have been described in APS and may confuse diagnosis. We reported a case of a middle-aged lady known to have APS, who presents with pathergy among cannulation and subsequent formation of nonhealing ulcers that have been relatively refractory to novel treatments. Currently, our patient is being trialed on a new biologic agent that has been proven to be efficacious as described recently in the literature.
