# Choroidal neovascularization secondary to angioid streaks in a patient with pseudoxanthoma elasticum: case report

**Authors:** David-Ionuț Beuran, Camelia Constantin, Răzvan Constantin Șerban, Lucian George Eftimie, Cătălin Cornăcel

PMC · DOI: 10.22336/rjo.2024.85 · Romanian Journal of Ophthalmology · 2024-10-01

## TL;DR

A 46-year-old patient with pseudoxanthoma elasticum developed choroidal neovascularization in the left eye, which was treated with aflibercept injections, improving vision.

## Contribution

This case report highlights the management of choroidal neovascularization in a patient with pseudoxanthoma elasticum.

## Key findings

- The patient showed angioid streaks and choroidal neovascularization in the left eye.
- Intravitreal aflibercept injections improved visual acuity in the affected eye.
- Pseudoxanthoma elasticum was confirmed via skin biopsy and histopathological analysis.

## Abstract

Present the diagnosis and therapeutic approach in a patient with pseudoxanthoma elasticum and ocular involvement.

A 46-year-old patient presented for progressive loss of vision and metamorphopsias in the left eye. The ophthalmological examination showed angioid streaks and secondary choroidal neovascularization. The dermatologist performed an incisional biopsy of a skin area in the right axilla that showed white-yellow, discrete streaks. Histopathological examination confirmed the diagnosis of pseudoxanthoma elasticum. It was decided to start intravitreal injections with aflibercept. Three injections were made in the left eye with subsequent visual acuity improvement.

Angioid streaks are a rare retinal condition, and pseudoxanthoma elasticum is the most common association. Other systemic conditions are Ehlers-Danlos syndrome, Paget’s disease, and hemoglobinopathies. Definite diagnosis requires skin lesions or calcified elastic fibers on pathological examination and angioid streaks of the retina. Choroidal neovascularization is a frequent complication and leads to vision loss. Intravitreal inhibitors of vascular endothelial growth factor are currently the first line of treatment.

Pseudoxanthoma elasticum has numerous systemic manifestations and requires a multidisciplinary team to be monitored and treated.

## Linked entities

- **Diseases:** pseudoxanthoma elasticum (MONDO:0009925), choroidal neovascularization (MONDO:0810000), angioid streaks (MONDO:0011782), Ehlers-Danlos syndrome (MONDO:0020066), Paget’s disease (MONDO:0021165)

## Full-text entities

- **Genes:** VEGFA (vascular endothelial growth factor A) [NCBI Gene 7422] {aka L-VEGF, MVCD1, VEGF, VPF}
- **Diseases:** Pseudoxanthoma elasticum (MESH:D011561), Angioid streaks (MESH:D000793), skin lesions (MESH:D012871), Paget's disease (MESH:C537701), loss of vision (MESH:D014786), Ehlers-Danlos syndrome (MESH:D004535), Choroidal neovascularization (MESH:D020256), hemoglobinopathies (MESH:D006453), retinal condition (MESH:D012164)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

20 references — full list in the complete paper: https://tomesphere.com/paper/PMC11809835/full.md

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Source: https://tomesphere.com/paper/PMC11809835