March 2012
Volume 53, Issue 14
Free
ARVO Annual Meeting Abstract  |   March 2012
Chronic Keratitis With Intrastromal Epitheliod Histiocytes: A New Finding In Muckle-wells Syndrome
Author Affiliations & Notes
  • Ian Gorovoy
    ophthalmology, UCSF, San Francisco, California
  • Mark Gorovoy
    ophthalmology, Gorovoy Eye Specialists, Fort Myers, Florida
  • Jaclyn Gorovoy
    1Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Florida
  • Footnotes
    Commercial Relationships  Ian Gorovoy, None; Mark Gorovoy, None; Jaclyn Gorovoy, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science March 2012, Vol.53, 1097. doi:
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    • Get Citation

      Ian Gorovoy, Mark Gorovoy, Jaclyn Gorovoy; Chronic Keratitis With Intrastromal Epitheliod Histiocytes: A New Finding In Muckle-wells Syndrome. Invest. Ophthalmol. Vis. Sci. 2012;53(14):1097.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: : To report a new corneal finding in Muckle-Wells syndrome (MWS) and its subsequent treatment.

Methods: : A 45 year-old woman with a history of MWS presented with progressive bilateral stromal 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 and trabeculectomy two years prior for open-angle glaucoma. The vision in the right eye was 20/40.

Results: : The patient underwent penetrating keratoplasty in the more severely affected left eye. Six months postoperatively the patient had a BSCVA of 20/40. Pathologic specimen demonstrated a chronic keratitis with focal calcification of Bowman’s layer, localized Descemet’s membrane breaks, marked endothelial loss, and pronounced intrastromal epitheliod histiocytes without evidence of amyloidosis.

Conclusions: : To the best of our knowledge, this case highlights a new association with MWS. Epitheliod histocytosis has been demonstrated in other parts of the body in patients with MWS including the skin and joints, although it is generally accompanied by amyloidosis.

Keywords: cornea: stroma and keratocytes • pathology: human • genetics 
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