Primary Periocular Orofacial Granulomatosis: Case Series and Systematic Review.

Purpose: There is no known optimal treatment for primary periocular orofacial granulomatosis (PPOFG), a disorder that results in periocular edema. This case series and systematic review identifies management strategies and their reported improvement.

Methods: Systematic review and case series. PubMed and MEDLINE databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines to identify published cases of PPOFG. Cases were included when edema involved the periocular tissues and when a biopsy was interpreted to be PPOFG. Cases were excluded when edema did not involve the periocular tissues or when the edema was secondary to another process. The electronic medical records of the pathology department were studied to identify cases that were in keeping with PPOFG. The clinical charts were examined to confirm the diagnosis and provide a local case series.

Results: There are 38 published cases of PPOFG. An additional 9 cases were identified locally. These cases were combined and analyzed. Most PPOFG has eyelid swelling in isolation, without other facial swelling (36/47; 76.6%). It is most commonly a bilateral disease (30/47; 63.8%). Fissured tongue and facial nerve palsy occur, just as they do in other cases of orofacial granulomatosis. Treatment with surgical debulking or intralesional steroids resulted in high rates of symptomatic improvement of eyelid swelling, but recurrences were common.

Conclusions: In light of no curative or highly successful treatment currently available, intralesional steroids and/or surgical debulking are therapies in the treatment of eyelid swelling associated with PPOFG that demonstrate reasonable short- and medium-term results. There is no established therapy that can offer disease remission or long-term symptom improvement.