Refractory postsurgical pyoderma gangrenosum in a patient with Beckwith Wiedemann syndrome: response to multimodal therapy

Abstract

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that may be difficult to diagnose and treat. We presented a 41-year-old woman who required skin grafting following third-degree burns to her left breast. She suffered recurrent graft dehiscence and infections over many years, prompting elective bilateral reduction mammoplasty. She subsequently developed suture margin ulcerations unresponsive to topical therapies and antibiotics. Skin biopsies were non-specific, and a clinical diagnosis of PG was established. Although initially responsive to corticosteroids, wounds promptly recurred following steroid taper. She was treated unsuccessfully with various immunomodulatory agents and underwent elective bilateral mastectomy. Following a mastectomy, she developed progressive deep chest wall ulcerations. She failed numerous immunomodulatory treatments, surgical wound closure and negative pressure wound therapy. Ultimately, treatment with adalimumab, mycophenolate mofetil and prednisone, in addition to hyperbaric oxygen therapy facilitated progressive healing. Our case highlights the role of collaborative multimodal therapy for the treatment of refractory PG.

Figure 1

Left breast ulcer biopsy. (A) Ulcerated, necrotic epidermis and intensely inflamed, focally necrotic dermis. (B) (High power) Marked acute and chronic inflammation and occasional giant cell formation. All cultures were negative.

Figure 2

Progression of left chest wall pyoderma gangrenosum wound healing, postmastectomy. (A) Wound measures 13×8×2 cm and the patient is taking prednisone 30 mg twice daily, mycophenolate mofetil (MMF) and just started adalimumab. (B) Nine months later, the patient is taking prednisone 7.5 mg daily, MMF, adalimumab and is completing hyperbaric oxygen therapy. (C) Another 5 months later, the patient is taking prednisone 15 mg daily, MMF and adalimumab. Wound is nearly closed, but with significant cribiform scarring.