PERIPHERAL ULCERATIVE KERATITIS SECONDARY TO PYODERMA GANGRENOSUM: A RARE AND VISION-THREATENING ASSOCIATION

To describe a rare case of bilateral peripheral ulcerative keratitis (PUK) associated with pyoderma gangrenosum (PG), an uncommon ocular manifestation of this neutrophilic dermatosis, highlighting the diagnostic and therapeutic challenges.

Department of Ophthalmology, Hospital de Santa Maria, Lisbon, Portugal.

A 47-year-old man with a history of pyoderma gangrenosum involving the skin and oropharyngeal mucosa since 2015, previously treated with corticosteroids, cyclosporine and biologic agents (infliximab and adalimumab), presented with progressive ocular pain and vision loss in June 2024. Ophthalmologic examination revealed severe bilateral peripheral ulcerative keratitis (PUK), more pronounced in the right eye, with a sterile hypopyon and no corneal infiltrates. Laboratory studies excluded systemic vasculitis or rheumatoid arthritis, with negative rheumatoid factor and ANCA.

Despite topical corticosteroids, systemic prednisone and cyclosporine, the patient developed progressive corneal thinning and eventually corneal perforation of the left eye in March 2025, followed by a tectonic penetrating keratoplasty with peritomy and amniotic membrane transplantation. Systemic therapy was optimized in coordination with Dermatology, including high-dose corticosteroids, doxycycline, and cyclosporine. Given the refractoriness to conventional immunosuppressants and prior anti-TNF intolerance, treatment with ustekinumab was initiated, achieving stabilization of both ocular and mucocutaneous disease. At 2-month follow-up, the graft remained clear, and no recurrent corneal melting was observed.

Peripheral ulcerative keratitis is an uncommon but severe ocular manifestation of pyoderma gangrenosum, often mimicking autoimmune vasculitides such as rheumatoid arthritis. Early recognition and aggressive systemic immunosuppression are crucial to prevent corneal perforation and irreversible visual loss. Multidisciplinary management involving ophthalmology, dermatology, and rheumatology is essential for optimal outcomes in such complex inflammatory disorders.