A 58-year-old man presented with sudden loss of vision in his left eye. The patient had been under follow-up by Internal Medicine and Dermatology for 12 years due to PXE.

His MAVC in the OD was 0.6 and in the OI 0.4. No alterations were found in IOP or anterior pole CMB.

The FO examination revealed bilateral AEs. In the OD, there was a raised yellowish-grey gliotic lesion and, in the OI, a pigmented triradiate image bordered by orange areas in the macular area, together with a small juxtafoveal lesion, somewhat greyish and slightly raised, surrounded by abundant subretinal haemorrhages, mainly nasal. Suspicion of CNV membrane within a "reticular pattern" retinal dystrophy led us to perform an AFG, where a gliotic eschar was observed in the OD and, in the OI, a hypofluorescent image in the form of a reticulum, in the upper area of which there was a hyperfluorescent lesion with lacy borders that increased in intensity and size in the late stages, thus confirming the previous suspicion. OCT corroborated the diagnosis, showing a discrete elevation of the EPR-choriocapillary band.

The FA of the LA revealed a curious image, as the dystrophy in pattern appeared as the negative of the angiographic image; in other words, the reticulum, which angiographically appeared hypofluorescent, was intensely hyper-FA in FA due to the alteration of the EPR at that level.

In this case, the same treatment as in the previous patient was applied according to the protocol described above. A higher VA was achieved, reaching 0.7. Progressive OCT scans showed a progressive decrease in macular thickness and the control AFG showed an improvement in the condition.