Pseudoxanthoma Elasticum — SCE Dermatology MCQ
Instant feedback + full explanation. One question, done properly.
Educational content. Not a substitute for clinical judgement or local policy.
Reveal the answer and explanation
Correct answer: C — Urgent retina review for intravitreal anti-VEGF treatment
Explanation lettering: D = shown as A · C = shown as B · A = shown as C · E = shown as D · B = shown as E
A is correct. Loss of vision in PXE is commonly driven by macular neovascularization at breaks in calcified Bruch membrane. GeneReviews recommends retina-specialist management with intraocular anti-angiogenic treatment when neovascularization is present. Angioid streaks alone are observed, but new metamorphopsia plus fluid and angiographic leakage marks an active sight-threatening complication. Panretinal laser treats retinal ischaemic neovascularization, not this subretinal process. Acetazolamide and systemic bisphosphonate do not reverse the inherited ABCC6 defect. Patients also need vascular-risk management and counselling about gastrointestinal bleeding risk.
Reference: GeneReviews: pseudoxanthoma elasticum: https://www.ncbi.nlm.nih.gov/books/NBK1113/