Cogan's syndrome with pyoderma gangrenosum: management of two uncommon disorders with aggressive presentation in a patient.

Pyoderma gangrenosum (PG) coexisting with Cogan's syndrome (CS) is uncommon, although cutaneous manifestations are known to develop in CS. A middleaged white female patient had chronic relapsing PG requiring ciclosporin and prednisolone. Despite receiving optimal doses of ciclosporin and prednisolon...

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Publicado en:BMJ Case Reports Vol. 2018; pp. 1 - 6
Autores principales: Pei-Chin Chua, Ezolene, Mallett, Richard B., Dahiya, Sandeep
Formato: Journal Article
Publicado: BMJ Publishing Group 6/19/2018
Acceso en línea:Ver este registro en EBSCOhost
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      dt: 6/19/2018
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      pub: BMJ Publishing Group
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        130309292
        10.1136/bcr-2017-223876
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        atl: Cogan's syndrome with pyoderma gangrenosum: management of two uncommon disorders with aggressive presentation in a patient.
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        au:
          Pei-Chin Chua, Ezolene
          Mallett, Richard B.
          Dahiya, Sandeep
        affil: Department of Rheumatology, Peterborough City Hospital, Peterborough, United Kingdom
      sug:
      ab: Pyoderma gangrenosum (PG) coexisting with Cogan's syndrome (CS) is uncommon, although cutaneous manifestations are known to develop in CS. A middleaged white female patient had chronic relapsing PG requiring ciclosporin and prednisolone. Despite receiving optimal doses of ciclosporin and prednisolone, she developed acute vestibulo-auditory symptoms as a result of CS. Ciclosporin was switched to methotrexate and prednisolone was increased. However, she continued to develop acute scleritis, requiring methylprednisolone pulses, and still had further flares of PG. Her methotrexate was held off when she developed severe pneumonia and she then received a trial of intravenous immunoglobulins (IVIG) for her recurrent leg ulcers. Unfortunately, she failed to respond to IVIG. Her ulcers eventually responded to six doses of monthly intravenous cyclophosphamide induction. Although CS is not an antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, we used pulse cyclophosphamide, based on the experience of cyclophosphamide efficacy in severe ANCA-associated vasculitis (AAV). Following induction, both diseases currently remain under control with azathioprine as maintenance treatment.
      pubtype: Academic Journal
      doctype: Journal Article
      ougenre: Article
    language: English
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