Acute Fibrinous Anterior Uveitis in a Patient Treated With Topical Interferon-α2b for Ocular Surface Squamous Neoplasia With Uveitis Susceptibility.

PURPOSE: To report a case of fibrinous acute anterior uveitis associated with topical interferon-α2b (IFN-α2b) treatment for ocular surface squamous neoplasia in a patient with HLA-B27 uveitis predisposition. METHODS: Case report. RESULTS: We present the case of a 57-year-old man who received topical IFN-α2b as adjuvant therapy for a previously surgically removed ocular surface squamous neoplasia with affected surgical margins. Two weeks after topical IFN-α2b initiation, the patient was diagnosed with fibrinous acute anterior uveitis. Complementary tests to rule out other causes of uveitis resulted to be negative, except for HLA-B27, which tested positive. Response to treatment with topical corticosteroids and cyclopentolate was favorable. As IFN-α2b is considered an immune enhancer and has been widely associated with autoimmune side effects, topical therapy with IFN-α2b was temporally ceased until intraocular inflammation resolved. Topical IFN-α2b was resumed, and during follow-up, no signs of uveitis were detected. The main hypothesis is that IFN-α2b acts as a trigger for intraocular inflammation in individuals with uveitis predisposition. CONCLUSIONS: Topical IFN-α2b could trigger intraocular inflammation in patients with uveitis susceptibility. It may be reasonable to use IFN-α2b cautiously in patients with a known history of uveitis or uveitis predisposition.

Posterior Polymorphous Corneal Dystrophy in a Patient with a Novel ZEB1 Gene Mutation.

Posterior polymorphous corneal dystrophy (PPCD), a rare, bilateral, autosomal-dominant, inherited corneal dystrophy, affects the Descemet membrane and corneal endothelium. We describe an unusual presentation of PPCD associated with a previously unknown genetic alteration in the ZEB1 gene. The proband is a 64-year-old woman diagnosed with keratoconus referred for a corneal endothelium study who presented endothelial lesions in both eyes suggestive of PPCD, corectopia and iridocorneal endothelial synechiae in the right eye and intrastromal segments in the left eye. The endothelial count was 825 in the right eye and 1361 in the left eye, with typical PPCD lesions visible under specular and confocal microscopy. In the next generation sequencing genetic analysis, a heterozygous c.1A > C (p.Met1Leu) mutation was found in the ZEB1 gene (TCF8). The PPCD3 subtype is associated with corneal ectasia, and both can appear due to a pathogenic mutation in the ZEB1 gene (OMIM #189909). However, our patient had a previously unreported mutation in the ZEB1 gene, which mediates the transition between cell lines and provides a pathogenic explanation for the epithelialisation of the corneal endothelium, a characteristic of PPCD.