Primary Periocular Orofacial Granulomatosis: Case Series and Systematic Review

Area of Science:

• Ophthalmology
• Dermatology
• Plastic Surgery

Background:

• Primary periocular orofacial granulomatosis (PPOFG) is a rare condition characterized by eyelid swelling.
• There is no established optimal treatment for PPOFG, necessitating the exploration of management strategies.

Approach:

• A systematic review of published cases and a local case series were conducted.
• Cases were identified through database searches and pathology records, adhering to PRISMA guidelines.
• Inclusion criteria focused on periocular edema confirmed by biopsy, excluding secondary causes.

Key Points:

• The study analyzed 47 cases of PPOFG, with most presenting isolated eyelid swelling (76.6%) and being bilateral (63.8%).
• Associated symptoms like fissured tongue and facial nerve palsy were noted.
• Surgical debulking and intralesional steroids showed high rates of symptomatic improvement for eyelid swelling.

Conclusions:

• Intralesional steroids and surgical debulking provide reasonable short- to medium-term results for PPOFG-related eyelid swelling.
• No current therapy offers disease remission or long-term symptom improvement for PPOFG.