Acute Posterior Multifocal Placoid Pigment Epitheliopathy with Granulomatous Uveitis Unmasking Crohn's Disease.

PURPOSE: To report a rare case of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) revealing Crohn's disease in an adolescent, and to discuss potential immunopathological links between these two inflammatory conditions.

METHODS

Case-Report.

RESULTS

A 16-year-old boy presented with a rapidly progressive bilateral central vision blurring. Examination revealed bilateral granulomatous hypertensive anterior uveitis, mild vitritis, and multifocal placoid lesions involving posterior poles. Multimodal imaging demonstrated active choriocapillaritis mimicking atypical APMPPE, with non-occlusive retinal vasculitis. Systemic evaluation, prompted by recent weight loss and digestive symptoms, led to a diagnosis of histologically confirmed Crohn's disease. Systemic corticosteroid therapy followed by anti-TNFα therapy (adalimumab) resulted in full remission, with no recurrence of inflammation or development of choroidal neovascularization after one year.

CONCLUSION

Although the exact pathophysiology of APMPPE remains uncertain, increasing evidence suggests an immune-mediated vasculitic mechanism rather than primary ischemia alone. This case is noteworthy as APMPPE revealed previously undiagnosed Crohn's disease. It highlights the need for systemic evaluation in atypical presentations, especially when granulomatous anterior involvement is present. Modern multimodal imaging, including OCT, provided precise characterization of the lesions and contributed to the comprehensive evaluation of this rare association.