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Year : 2021  |  Volume : 33  |  Issue : 2  |  Page : 211-213

Pseudoxanthoma elasticum: Pathognomonic ocular and skin lesions with histopathological confirmation

1 Department of Vitreo-retina Services, Sri Sai Aravind Eye Hospital, Hosapete, Karnataka, India
2 Department of Vitreo-retina Services, Aravind Eye Hospital, Madurai, Tamil Nadu, India
3 Department of Ophthalmology, JIPMER, Puducherry, India
4 Department of Pathology, Aravind Eye Hospital, Madurai, Tamil Nadu, India

Correspondence Address:
Dr. Amit Kumar Deb
Department of Ophthalmology, Old IPD Block, 1st Floor, JIPMER, Gorimedu, Puducherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_98_20

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A 34-year-old female presented to us with complaints of decreased vision in her left eye for 1 month. Best-corrected visual acuity was 20/20 in the right eye and 20/60 in the left eye. Anterior segment examination was normal in both the eyes. Fundus examination showed angioid streaks and comet tail lesions in both eyes. The left eye in addition showed a choroidal neovascular membrane (CNVM) at the macula. Systemic examination revealed skin lesions on the neck with a plucked chicken appearance and multiple papules. Biopsy from the lesions provided histopathological confirmation of pseudoxanthoma elasticum (PXE). CNVM in the left eye was treated successfully with a single dose of intravitreal bevacizumab injection with no further recurrence. No cardiovascular association was found in our case. This case report describes the pathognomonic fundus lesions seen in PXE. This case also emphasizes the need for thorough systemic evaluation, including biopsy from skin lesions for histopathological confirmation of the diagnosis in all cases with ocular features of PXE.

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