A 52-year-old woman came to the emergency department for sudden and painless loss of vision in the left eye (OI). She had no relevant personal or family history.
On ophthalmological examination, her best corrected visual acuity (BCVA) in the right eye (RA) was 0.9 and in the LA 0.6. Intraocular pressure (IOP) and biomicroscopy (BMC) of the anterior pole showed no significant alterations. Fundus visualisation (FO) showed a brownish mottling, more prominent temporal to the fovea, corresponding to the typical "orange peel fundus" image. There were also characteristic angioid striae (AE), as well as colloid bodies in both eyes (AO). In his OI, there were striking drusen of the optic nerve and a subfoveal greyish lesion within an elevated AE, bordered by haemorrhages, compatible with CNV.

A series of complementary tests were therefore performed, showing diffuse hyperautofluorescent mottling (hyper-FA) and irregular linear hypoautofluorescent lesions (hypo-FA) with hyper-FA mottling in their bed in the fundus autofluorescence (FA) of the AO. In the OI, optic nerve drusen and colloid bodies with intense hyper-FA were prominent.
Fluorescein angiography (FAG) revealed a hyper- and hypofluorescent lattice due to alteration of the retinal pigment epithelium (RPE) and a hyperfluorescent lesion with late diffusion in the LA compatible with CNV.
Optical coherence tomography (OCT) showed a destructuring of the EPR-choriocapillary hyperreflective band in the AO, and in the OI a dense cupuliform lesion (CNV), bordered by a small detachment of neuroepithelium.
After ophthalmological diagnosis, she was referred to the elastic pseudoxanthoma unit of our hospital, where the disease was confirmed.
Treatment was prescribed with monthly intravitreal injections of 0.05ml of ranibizumab (Lucentis) for 3 months in her OI, with measurement of the MAVC and OCT one month after each injection, and control AFG at 3 months. Periodic follow-up OCT scans were performed for a follow-up period of one year. At 3 months, a clear improvement in the condition was observed, as the MAVC improved to 0.7, subretinal haemorrhages disappeared and macular thickness decreased on OCT.