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.
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.
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.1 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.1, 2, 3, 4 Rose and colleagues5 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.5 A known cutaneous manifestation of GPA, pyoderma gangrenosum-like lesions can herald diagnosis.6 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.
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-Michel J. Belliveau, MD, FRCS, Baruch D. Jakubovic, MD, Dharini Mahendira, MD, FRCPC, Henry R. Jakubovic, MD, FRCPC, Navdeep Nijhawan, MD, FRCSC
This article originally appeared in the February 2016 issue of The American Journal of Medicine.
