Primary Periocular Orofacial Granulomatosis
Authors: David Plemel, Ezekiel Weis, Karim Punja, Michael Ashenhurst, Andrzej Kulaga.
Author Disclosure Block: D. Plemel: None. E. Weis: None. K. Punja: Membership on advisory boards or speakers’ bureaus; Name of for-profit or not-for-profit organization(s); Alcon Canada Inc, Allergan Inc, Clarion Medical Technologies. Membership on advisory boards or speakers’ bureaus; Description of relationship(s); Consultant / Advisor. M. Ashenhurst: None. A. Kulaga: None.
Purpose: The purpose of this study was to describe the management of primary periocular orofacial granulomatosis (OFG).
Study Design: Case series and systematic review.
Methods: The electronic records of the Department of Laboratory Medicine and Pathology at the University of Calgary identified all cases of possible periocular OFG. Clinical charts were reviewed to identify those that were in keeping with the diagnosis of primary periocular OFG. A literature review was completed using PRISMA guidelines.
Results: Twelve cases of primary periocular OFG were identified locally. Forty-four additional cases were identified through literature review. The combined 55 cases were analyzed. Alone or in combination with other treatments, intralesional steroids (100%; 19/19), surgical debulking (94.4%; 17/18) and systemic steroids (72.2%; 13/18) show the best evidence for treatment success. Although some management strategies improved disease burden, there were relapses in nearly all cases.
Conclusions: Primary periocular OFG is rare, and treatment is based on expert opinion. Surgical debulking with ongoing intralesional steroids is a commonly used method. However, relapses are nearly universal as with all known treatment modalities. Effective systemic therapies remain elusive.