Primary Periocular Orofacial Granulomatosis G51.2

Rare, etiologically unexplained clinical picture with initially recurrent, later persistent bilateral swelling of the eyelids.

Most cases of PPOFG are bilateral swellings limited to the eyelids without other swellings on the face. As with other orofacial granulomatoses (in the context of Melkersson-Rosenthal syndrome), lingua plicata and facial nerve palsies may also occur (Plemel, D et al. 2024).

As no curative or highly successful treatment is currently available, intralesional steroids and/or surgical debulking are therapies for the treatment of eyelid swelling associated with PPOFG that show reasonable short- and medium-term results.

Treatment with surgical debulking or intralesional steroids resulted in a high rate of symptomatic improvement of eyelid swelling. Recurrences are possible.

There are 38 published cases of PPOFG to date (Plemel, D et al. 2024).

1. Plemel, D et al. (2024) Primary Periocular Orofacial Granulomatosis: Case Series and Systematic Review. Ophthalmic Plastic and Reconstructive Surgery 40: 11-17.