Wells Syndrome as a Rare Cause of Unilateral Ptosis

Abstract

We present a case of a woman with a swollen upper eyelid in ptosis, conjunctival nodules, and chemosis present for a couple of weeks. She did not respond to therapy for hordeolum or allergy. A biopsy followed by histopathological examination showed a large infiltration of eosinophilic granulocytes and flame figures, so the diagnosis of Wells syndrome was made. Oral methylprednisolone in high dose was necessary, and tapering was slow because of frequent bouts of relapse. After 3 months, the swelling and hard feeling of the eyelid disappeared, but a slight ptosis remained. Eyelid swelling and eosinophilia recurred 18 months after resolution of the first episode.

Wells Syndrome Presenting as Atypical Periorbital Cellulitis

“…Among these, orbital involvement is one of the most commonly reported findings. 8,9 To our knowledge, we present the first reported case of an orbital silicone implant exposure in a patient with an undiagnosed GPA at presentation. The patient consented to publication and archival of identifiable photographs.…”

“…6 Facial lesions secondary to Wells syndrome are exceedingly rare. 4,5,[7][8][9] Of the 8 reported cases in the literature, none were sight-threatening. To the authors' knowledge, this is the first reported case of severe periorbital Wells syndrome causing acute decline in vision requiring urgent intervention.…”

“…5 Ocular involvement is found in up to 60% of patients and can occur as the first presenting feature of GPA before progression into multisystem involvement. [6][7][8][9] Clinical manifestations include tarsal conjunctival disease, episcleritis, scleritis, keratitis, granulomatous sclerouveitis, retinal vasculitis, nasolacrimal obstruction, and orbit/adnexal disease. Among these, orbital involvement is one of the most commonly reported findings.…”

“…[1][2][3][4][5] Facial or periorbital involvement is exceedingly rare and remains limited to a few case reports. [6][7][8][9] Herein, the authors report a case of unilateral, painless periorbital edema and erythema, and eosinophilic tissue infiltration that was most consistent with Wells syndrome. To the best of the authors' knowledge, this is the first reported case of Wells syndrome causing acute vision loss and elevated intraocular pressure (IOP).…”