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

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 elec...

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Publicado en:BMJ Case Reports pp. 1 - 5
Autores principales: Fakhar, Faiza, Memon, Sehrish, Deitz, Diane, Abramowitz, Richard, Alpert, Deborah R.
Formato: case study pictorial Journal Article
Publicado: BMJ Publishing Group Oct2013
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Oct2013
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      pub: BMJ Publishing Group
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        91950888
        10.1136/bcr-2013-201084
        104116316
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        atl: Refractory postsurgical pyoderma gangrenosum in a patient with Beckwith Wiedemann syndrome: response to multimodal therapy.
      aug:
        au:
          Fakhar, Faiza
          Memon, Sehrish
          Deitz, Diane
          Abramowitz, Richard
          Alpert, Deborah R.
        affil: Department of Internal Medicine, Jersey Shore University Medical Center, Neptune, New Jersey, USA
      sug:
        subj:
          Beckwith-Wiedemann Syndrome Diagnosis
          Combined Modality Therapy
          Pyoderma Gangrenosum Therapy
          Treatment Outcomes
          Breast Reconstruction Adverse Effects
          Female
          Adult
          Staining and Labeling
          Diagnosis, Differential
          Adult: 19-44 years
          Female
      ab: 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.
      pubtype: Academic Journal
      doctype:
        case study
        pictorial
        Journal Article
      ougenre: Article
    language: English
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