title: 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.
authors.affiliation: Department of Internal Medicine, Jersey Shore University Medical Center, Neptune, New Jersey, USA.
authors.forename: Faiza
authors.initials: F
authors.lastname: Fakhar
authors.forename: Sehrish
authors.initials: S
authors.lastname: Memon
authors.forename: Diane
authors.initials: D
authors.lastname: Deitz
authors.forename: Richard
authors.initials: R
authors.lastname: Abramowitz
authors.forename: Deborah R
authors.initials: DR
authors.lastname: Alpert
mesh_terms: D000068879:Adalimumab; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D001940:Breast / Q000293:injuries / Q000601:surgery; D002056:Burns / Q000601:surgery; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D017511:Pyoderma Gangrenosum / Q000209:etiology* / Q000628:therapy
chemical_list: D000893:Anti-Inflammatory Agents; D061067:Antibodies, Monoclonal, Humanized; D007166:Immunosuppressive Agents; D000068879:Adalimumab; D009173:Mycophenolic Acid; D011241:Prednisone