If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
Requests for reprints should be addressed to Michel J. Belliveau, MD, FRCSC, Department of Ophthalmology & Vision Sciences, University of Toronto, 1503-832 Bay St., Toronto, ON M5S 1Z6, Canada.
Department of Medicine, University of Toronto, St. Michael's Hospital, Toronto, ON, CanadaDivision of Rheumatology, University of Toronto, St. Michael's Hospital, Toronto, ON, Canada
A man in his late fifties sought care in the Emergency Department for expanding redness and increasing discharge from his right upper eyelid. The symptoms were preceded by the presence of a “stye” for a few weeks. It had been squeezed until it “popped,” and subsequently re-formed before evolving into an oozing tract. The patient lived and worked on a poultry farm, and frequently performed activities that put him at risk for penetrating cutaneous foreign bodies.
Assessment
The redness and discharge had been refractory to oral cephalosporin and subsequent trials of intravenous cephalosporin, clindamycin, and piperacillin/tazobactam. Our examination revealed a severely inflamed right upper eyelid with copious purulent discharge and tissue loss (Figure 1).
Figure 1Severely inflamed right upper eyelid with purulent discharge. There is loss of the distal portion of the anterior lamella beyond the eyelid crease over the length of the eyelid, with sparing of the eyelashes and margin. Full-thickness upper eyelid loss is seen in the temporal third. The wound edge is undermined and has an elevated violaceous border.
The ocular surface was inflamed with initial sparing of the cornea and 20/30 best-corrected visual acuity. There were no signs of intraocular or orbital involvement. A computed tomography study was negative for abscess formation and postseptal orbital involvement. However, the radiologist reported the presence of a foreign body. The wound was irrigated copiously and explored. No foreign matter was visible. Tissue culture and biopsy were obtained. Systemic investigations were performed in this setting of grossly necrotizing inflammation. A neutrophilic leukocytosis was detected and acute phase reactants were elevated. Antineutrophil cytoplasmic antibodies to proteinase 3 were strongly positive, highly specific for granulomatosis with polyangiitis (GPA). Histopathological analysis revealed necrotizing granulomatous inflammation without vasculitis and neutrophilic collections in the superficial and deep dermis (Figure 2).
Figure 2Light microscopic image of the superficial dermis showing a mixed inflammatory infiltrate with abundant histiocytes and a neutrophilic collection in a focus of necrosis (hematoxylin-eosin, original magnification ×200).
Tissue culture and special stains for microorganisms were negative for bacteria, mycobacteria, and fungi. Creatinine and urinalysis were normal. Computed tomography of the chest showed multiple bilateral pulmonary nodules also consistent with GPA.
Diagnosis
The most immediate concern in the setting of eyelid necrosis is periorbital necrotizing fasciitis, which can be blinding and fatal.
The evolution of this man's presentation did not match the rapid tempo seen with necrotizing fasciitis, and we were reluctant to debride the eyelid. Foreign bodies retained in the eyelid or deeper in the orbit can present with a fistulous tract, and the radiologic interpretation in this case steered us in that direction briefly. It is unclear what caused the computed tomographic appearance of a foreign body, but repeat imaging was negative following wound irrigation.
A variety of causes of eyelid necrosis have been reported, including infectious, traumatic, and iatrogenic etiologies.
reported a series of 4 female patients with periocular pyoderma gangrenosum that shared many features of the present case. Swelling progressed to frank necrosis and full-thickness eyelid loss. There was a predilection for temporal loss in the lower eyelid, which we observed in the upper eyelid. There was sparing of the eyelashes and lid margin where involvement was not full thickness, a finding seen in other etiologies, and presumably a result of a preserved marginal artery. Antineutrophil cytoplasmic antibody-associated vasculitis was not reported in their 4 patients.
Although a rare occurrence, pyoderma gangrenosum and GPA should be considered in the setting of eyelid necrosis, as highlighted by this case.
Management
The patient received induction treatment with pulsed intravenous methylprednisolone and cyclophosphamide. Before disease control was achieved, he suffered a corneal melt from peripheral ulcerative sclerokeratitis and ocular surface exposure, requiring corneal transplantation. Azathioprine and low-dose prednisone have maintained clinical and serological quiescence for 2 years.
Aimee K. Zaas, MD, Thomas J. Marrie, MD, Section Editors
Footnotes
Funding: There was no funding of this work.
Conflict of Interest: There are no conflicts of interest or financial/proprietary disclosure for any author.
Authorship: All authors contributed substantially—conception (MB, BJ, NN), acquisition and analysis of data (MB, BJ, DM, HJ, NN)—and guarantee the work. Drafted by MB, revised by BJ, DM, HJ, NN, and approved by all.
00906-7&id=gr1.jpg){kind=link}
00906-7&id=gr2.jpg){kind=link}