Angioid Streaks Revealing Pseudoxanthoma Elasticum: Ophthalmic Findings Leading to Dermatological Diagnosis
A. Hajjaji *
Department of Ophthalmology, Moulay Ismail Military Hospital, Meknes, Morocco.
S. Sadiki
Department of Ophthalmology, Military Hospital Mohammed V, Rabat, Morocco.
A. Bouzidi
Department of Ophthalmology, Moulay Ismail Military Hospital, Meknes, Morocco.
A. El Ouafi
Department of Ophthalmology, Moulay Ismail Military Hospital, Meknes, Morocco.
*Author to whom correspondence should be addressed.
Abstract
Background: Pseudoxanthoma elasticum (PXE) is a rare inherited multisystem disorder characterised by progressive calcification of elastic tissues. Angioid streaks may provide an important ophthalmic clue, prompting investigation for associated cutaneous and systemic manifestations.
Purpose: To describe a case in which the identification of angioid streaks during ophthalmic examination prompted dermatological assessment and histopathological confirmation of PXE.
Case Presentation: A 60-year-old woman presented with blurred vision in both eyes. Best-corrected visual acuity was 8/10 in both eyes. Anterior segment examination and intraocular pressure were normal. Fundus examination identified angioid streaks, more prominent in the right eye, and fundus autofluorescence demonstrated corresponding hypoautofluorescent fissures. Clinical examination showed no evidence of macular haemorrhage, exudation or choroidal neovascularisation. A suspicious lesion on the neck prompted referral for dermatological assessment. Examination revealed yellowish papules coalescing into plaques with a characteristic “plucked chicken skin” appearance. Skin biopsy and histopathological examination confirmed PXE. Cardiovascular assessment, including clinical examination, blood pressure measurement and echocardiography, yielded normal findings. The patient was educated about Amsler grid self-monitoring, seeking prompt consultation for new metamorphopsia or sudden visual loss, and avoiding ocular trauma. Regular follow-up using optical coherence tomography was planned.
Conclusion: This case illustrates how ophthalmic findings may contribute to the recognition of PXE when cutaneous manifestations have not previously led to its diagnosis. Although no clinical neovascular complication was detected at presentation, continued ophthalmic surveillance and multidisciplinary assessment are appropriate. The observations document one diagnostic pathway and do not establish its frequency or the patient's subsequent clinical course.
Keywords: Pseudoxanthoma elasticum, angioid streaks, fundus autofluorescence, dermatological diagnosis, Bruch’s membrane, skin biopsy