title: Chronic keratitis with intrastromal epithelioid histiocytes: a new finding in Muckle-Wells syndrome.
abstract: OBJECTIVE
  To report a new corneal finding in Muckle-Wells syndrome (MWS) and its subsequent treatment.
  
  
  METHODS
  The medical chart of a 45-year-old woman with a history of MWS was reviewed. She presented with progressive bilateral stromal edema and scarring that had been worsening over the past year. The left eye, which had more severe stromal scarring and a best spectacle-corrected visual acuity (BSCVA) of hand motion, had a history of cataract removal with lens implantation, argon laser trabeculoplasty, and trabeculectomy 2 years previously for open-angle glaucoma. The right eye had also undergone argon laser trabeculoplasty and trabeculectomy and had a BSCVA of 20/100.
  
  
  RESULTS
  The patient underwent penetrating keratoplasty in the more severely affected left eye. Nine months postoperatively the patient had a BSCVA of 20/40 in the eye that had undergone transplantation. The pathologic specimen demonstrated a chronic keratitis with focal calcification of Bowman layer, localized Descemet membrane breaks, marked endothelial loss, and pronounced intrastromal epithelioid histiocytes without evidence of amyloidosis.
  
  
  CONCLUSIONS
  To the best of our knowledge, this case highlights a new association with MWS. Epithelioid histiocytes have been demonstrated in other parts of the body in patients with MWS including the skin and joints, which is sometimes accompanied by amyloidosis.
authors.affiliation: Department of Ophthalmology, University of California, San Francisco, San Francisco, CA, USA.
authors.forename: Ian R
authors.initials: IR
authors.lastname: Gorovoy
authors.forename: Jaclyn B
authors.initials: JB
authors.lastname: Gorovoy
authors.forename: Diva
authors.initials: D
authors.lastname: Salomao
authors.forename: Mark S
authors.initials: MS
authors.lastname: Gorovoy
mesh_terms: D002908:Chronic Disease; D056587:Cryopyrin-Associated Periodic Syndromes / Q000150:complications*; D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D006644:Histiocytes / Q000473:pathology*; D006801:Humans; D007634:Keratitis / Q000473:pathology*; D008875:Middle Aged