title: Malignant Pyoderma Associated with Granulomatosis with Polyangiitis (Wegener Granulomatosis) as a Unique Indication for Facial Vascularized Composite Allotransplantation: Part I.
abstract: BACKGROUND
  Granulomatosis with polyangiitis (Wegener granulomatosis) is a rare disease that commonly starts in the craniofacial region and can lead to considerable facial disfigurement. Granulomas and vasculitis, however, can involve many other tissues (especially pulmonary and renal). Dermatologic and subcutaneous components can lead to malignant pyoderma.
  
  
  METHODS
  The authors describe a unique pathologic condition, where significant Le Fort type trauma was associated with subsequent development of granulomatosis with polyangiitis and malignant pyoderma. Successive operations to excise necrotic tissue and reconstruct the defects were followed by worsening inflammation and tissue erosions. Trauma and surgery in proximity to the eye and sinuses masked the initial clinical presentation and led to delay in diagnosis and disease progression. The resultant facial disfigurement and tissue loss were substantial.
  
  
  RESULTS
  Despite multiple confounding factors, accurate diagnosis was eventually established. This was based on persistence of sinus inflammations in the absence of infective agents, proven sterility of lung lesions, and antineutrophil cytoplasmic antibody positivity with proteinase 3 specificity. Skin lesion biopsy specimens were identified as pyoderma gangrenosum and later as malignant pyoderma. Institution of immunosuppressive therapy allowed successful control of the disease and wound healing. The resulting craniofacial destruction, however, necessitated facial vascularized composite allotransplantation.
  
  
  CONCLUSIONS
  Recognition of this rare pathologic association is essential, to prevent delays in diagnosis and treatment that can lead to major craniofacial tissue loss.
  
  
  METHODS
  Therapeutic, V.
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Brian
authors.initials: B
authors.lastname: Gastman
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Ahmed M
authors.initials: AM
authors.lastname: Hashem
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Risal
authors.initials: R
authors.lastname: Djohan
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Steven
authors.initials: S
authors.lastname: Bernard
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Mark
authors.initials: M
authors.lastname: Hendrickson
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Graham
authors.initials: G
authors.lastname: Schwarz
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Bahar B
authors.initials: BB
authors.lastname: Gharb
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Antonio
authors.initials: A
authors.lastname: Rampazzo
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Anthony
authors.initials: A
authors.lastname: Fernandez
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: James
authors.initials: J
authors.lastname: Zins
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Gary S
authors.initials: GS
authors.lastname: Hoffman
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Gaby
authors.initials: G
authors.lastname: Doumit
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Maria
authors.initials: M
authors.lastname: Siemionow
authors.affiliation: Cleveland, Ohio; and Cairo, Egypt.|From the Cleveland Clinic and Cairo University.
authors.forename: Francis
authors.initials: F
authors.lastname: Papay
mesh_terms: D000328:Adult; D064591:Allografts; D003131:Combined Modality Therapy; D064595:Composite Tissue Allografts / Q000601:surgery*; D003646:Debridement; D003937:Diagnosis, Differential; D018450:Disease Progression; D054445:Facial Transplantation / Q000379:methods*; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D008297:Male; D008440:Maxillary Fractures / Q000150:complications* / Q000175:diagnosis / Q000601:surgery*; D017551:Microdialysis; D011183:Postoperative Complications / Q000175:diagnosis / Q000601:surgery*; D011711:Pyoderma / Q000601:surgery*; D012086:Reoperation