Pseudoxanthoma elasticum medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayokunle Olubaniyi, M.B,B.S [2]

Overview

There is no specific treatment for PXE. Treatment focuses on prevention, screening and management of complications. The ocular complications can be managed with laser photocoagulation, transpupillary thermotherapy, photodynamic therapy or with anti-VEGF therapy. Pentoxifylline has been used to reduce the blood viscousity. Cardiovascular risk factors can be reduced by the avoidance of smoking, diet and exercising.

Medical Therapy

Currently, there is no specific or effective treatment available for PXE. The management focuses on prevention, screening, and management of complications.

Ocular complications

Treatment is indicated when choroidal neovascularisation (CNV) developes.

• Laser photocoagulation - Photocoagulation using light (xenon) and argon has been used with discouraging results in the past. Research reveals that laser photocoagulation for macular CNV in association with angioid streaks is of limited application due to the high percentage of neovascular membrane relapse.
• Transpupillary thermotherapy (TTT) - This involves the delivery of heat through the dilated pupil into the posterior segment of the eye. This therapy may decrease the activity of CNV in the short term but retreatment may be necessary with unfavourable results.
• Photodynamic therapy - This involves the use of verteporfin for the treatment of CNV in the macular. This method has not been widely accepted due to its inability to improve the natural course of CNV in angioid streaks.
• Anti-VEGF therapy - Intravitreous injection of vascular endothelial growth factor antagonists such as Ranibizumab, Bevacizumab have been used with excellent results.
• Vitamins A, C, E and zinc supplements may reduce the risk of bleeding.

Cardiovascular complications

• Diet and exercise - These are the main methods to minimize the extent of cardiovascular disease. Exercises help in weight reduction and it stimulates collateral blood vessel development.
• Pentoxifylline - This drug alters erythrocyte rheology, thus, reducing the blood viscosity. This drug should be used with caution due to its ability to cause hemorrhage.
• Cessation of tobacco - Tobacco worsens the outcome of this condition.

References

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