A Case Series of Patients Diagnosed With Orofacial Granulomatosis Presenting Primarily With Dense Infiltrates and Severe Periorbital Edema

Abstract

Orofacial granulomatosis is a relapsing nonnecrotizing granulomatous syndrome that classically presents with lip and perioral swelling. Over the years, several patients have been referred to the Duke Eye Center Oculoplastics Department for severe, progressive, recurrent eyelid swelling interfering with both their functional vision and their appearance. In this IRB approved retrospective case series, we describe the clinical course of 5 such patients, including their presenting symptoms, diagnosis, and response to treatment. We hope that oculoplastics specialists will consider this entity in the differential diagnosis of periorbital edema and consider initiating localized anti-inflammatory treatment once the diagnosis has been made.

The mouth in inflammatory bowel disease and aspects of orofacial granulomatosis

“…Orofacial granulomatosis can also present as recurrent facial swelling, with or without erythema, which may affect the lips, cheeks, eyelids, and forehead. Swelling of the upper half of the face may rarely occur and may include periorbital oedema …”

Orbital inflammatory disease management

“…In a recent case series by Sabet-Peyman et al, patients with orofacial granulomatosis, a disease closely related to MRS, found that local injections of triamcinolone and 5-fluorouracil improved eyelid edema and allowed for steroid tapering in some patients [228]. In MRS patients with recurrent or progressive paralysis of the facial nerve, facial nerve decompression via a transmastoid approach has been shown to improve paralysis [230].…”

“…Treatment for MRS leading to eyelid edema typically involves corticosteroids, and resolution of edema has been reported with both intralesional and systemic administration [225][226][227][228][229], although improvement is often short-lived given the relapsing and remitting nature of the disease.…”

“…Within the orbit and adnexa, MRS usually presents with upper and lower eyelid edema [224][225][226][227][228][229].…”

Atypical Orofacial Granulomatosis Associated With Bowel Relapse in an Adult With Crohn’s Disease Under Anti-TNF Therapy

“…In rare cases, OFG can involve other parts of the face, such as the cheeks and forehead. 2 Several treatments have shown variable results (exclusive diet, antibiotics, intralesional or systemic corticosteroids, and other immunomodulating therapies). Our patient responded well to doxycycline and relapsed when it was interrupted despite infliximab dose escalation, suggesting an independent effect of the antibiotic on OFG.…”