Chronic Keratitis with Intrastromal Epithelioid Histiocytes: A New Finding in Muckle–Wells Syndrome

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Purpose: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.

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