Multimodal therapy of idiopathic pyoderma gangrenosum

A 13-year old girl was admitted to the University of California Davis Medical Center for evaluation and treatment of cutaneous bullae and ulcerations over her lower extremities that were refractory to antibiotic therapy and incision and drainage. Her disease continued to worsen with the appearance of multiple new bullae and the progression of old ones into deep ulcers with undermined borders. Biopsy revealed a neutrophilic dermatosis and diagnostic work-up was negative for infectious or autoimmune etiologies. Given her clinical presentation, biopsy results, and negative work-up, a diagnosis of pyoderma gangrenosum (PG) was made and she was started on immunosuppressive medications. The patient was started on a multidrug regimen of prednisone and cyclosporine but remission was not achieved until the addition of adalimumab. After the inflammatory component of her disease was under control, wound care measures were maximized to promote ulcer healing. Wound care measures included compression and debridement. Upon complete closure of all wounds she was successfully transitioned to mycophenolate mofetil monotherapy for maintenance therapy. This case emphasizes the need for combinational therapy to successfully treat severe cases of PG, which are often refractory to monotherapy with prednisone or cyclosporine. It also highlights the importance of appropriate wound care to achieve complete ulcer healing.