eMedicine Specialties > Ophthalmology > Connective Tissue Disorders

Pseudoxanthoma Elasticum: Follow-up

Author: Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Coauthor(s): Diego Calonje, MD, Consulting Staff, Department of Ophthalmology, Private Practice; Sherif M El-Harazi, MD, MPH, Consulting Staff, Department of Ophthalmology, Sherif El-Harazi, MD
Contributor Information and Disclosures

Updated: Nov 18, 2008

Follow-up

Further Outpatient Care

  • Hypertension should be monitored.
  • Patients should receive regular eye examinations.

Deterrence/Prevention

  • Pregnancy, oral contraceptives, and high calcium and phosphorus intake seem to aggravate all manifestations of pseudoxanthoma elasticum.

Complications

  • Blindness
  • Recurrent GI hemorrhage
  • Myocardial infarction
  • Subarachnoid hemorrhage
  • Cerebrovascular accidents
  • Death

Prognosis

  • The greatest morbidity in patients with pseudoxanthoma elasticum and angioid streaks is the loss of central vision that occurs in a high percentage of patients in the fifth to sixth decade of life.

Patient Education

  • Inform and educate patients about the multisystem potential complications of pseudoxanthoma elasticum.

Miscellaneous

Medicolegal Pitfalls

  • Failure to recognize associated conditions, such as subarachnoid hemorrhage, upper GI hemorrhage, and decrease in visual acuity, and failure to inform patients of the possibility of the occurrence of these conditions.

Special Concerns

  • Careful evaluation of the patient and a detailed dilated eye examination of the ocular fundus aids in establishing the diagnosis because angioid streaks are eventually present in almost all the cases.
 


More on Pseudoxanthoma Elasticum

Overview: Pseudoxanthoma Elasticum
Differential Diagnoses & Workup: Pseudoxanthoma Elasticum
Treatment & Medication: Pseudoxanthoma Elasticum
Follow-up: Pseudoxanthoma Elasticum
References
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References

  1. Finger RP, Charbel Issa P, Ladewig M, et al. Intravitreal bevacizumab for choroidal neovascularisation associated with pseudoxanthoma elasticum. Br J Ophthalmol. Apr 2008;92(4):483-7. [Medline].

  2. Agarwal A, Patel P, Adkins T, et al. Spectrum of pattern dystrophy in pseudoxanthoma elasticum. Arch Ophthalmol. Jul 2005;123(7):923-8. [Medline].

  3. Albert DM, et al. Pseudoxanthoma elasticum (PXE)-angioid streaks/systemic associations. In: Principles and Practice of Ophthalmology Clinical Practice. Philadelphia: WB Saunders; 1994.

  4. Audo I, Vanakker OM, Smith A, et al. Pseudoxanthoma elasticum with generalized retinal dysfunction, a common finding?. Invest Ophthalmol Vis Sci. Sep 2007;48(9):4250-6. [Medline].

  5. Chung AK, Gauba V, Ghanchi FD. Photodynamic therapy (PDT) using verteporfin for juxtafoveal choroidal neovascularisation (CNV) in angioid streaks (AS) associated with pseudoxanthoma elasticum: 40 months results. Eye. May 2006;20(5):629-31. [Medline].

  6. Clarkson JG, Altman RD. Angioid streaks. Surv Ophthalmol. Mar-Apr 1982;26(5):235-46. [Medline].

  7. Coleman K, Ross MH, Mc Cabe M, et al. Disk drusen and angioid streaks in pseudoxanthoma elasticum. Am J Ophthalmol. Aug 15 1991;112(2):166-70. [Medline].

  8. Connor PJ Jr, et al. Pseudo-xanthoma elasticum and angioid streaks: a review of 106 cases. Am J Med. 1961;30:537-43.

  9. Engelman MW, Fliegelman MT. Pseudoxanthoma elasticum. Cutis. Jun 1978;21(6):837-40. [Medline].

  10. Goodman RM, et al. Pseudoxanthoma elasticum: A clinical and histopathological study. Medicine. 1963;42:297.

  11. Grand MG, Isserman MJ, Miller CW. Angioid streaks associated with pseudoxanthoma elasticum in a 13-year-old patient. Ophthalmology. Feb 1987;94(2):197-200. [Medline].

  12. Iqbal A, Alter M, Lee SH. Pseudoxanthoma elasticum: a review of neurological complications. Ann Neurol. Jul 1978;4(1):18-20. [Medline].

  13. Martinez-Hernandez A, Huffer WE. Pseudoxanthoma elasticum: dermal polyanions and the mineralization of elastic fibers. Lab Invest. Aug 1974;31(2):181-6. [Medline].

  14. Sawa M, Ober MD, Freund KB, et al. Fundus autofluorescence in patients with pseudoxanthoma elasticum. Ophthalmology. May 2006;113(5):814-20.e2. [Medline].

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Further Reading

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Keywords

pseudoxanthoma elasticum, PXE, Grönblad-Strandberg syndrome, peau d orange, peau d’orange, OMIM# 177850, OMIM# 264800, connective tissue disorder, calcification and fragmentation of elastic fibers, connective-tissue disorders

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Contributor Information and Disclosures

Author

Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Coauthor(s)

Diego Calonje, MD, Consulting Staff, Department of Ophthalmology, Private Practice
Disclosure: Nothing to disclose.

Sherif M El-Harazi, MD, MPH, Consulting Staff, Department of Ophthalmology, Sherif El-Harazi, MD
Sherif M El-Harazi, MD, MPH is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, and International Society of Refractive Surgery
Disclosure: Nothing to disclose.

Medical Editor

V Al Pakalnis, MD, PhD, Professor of Ophthalmology, University of South Carolina School of Medicine; Chief of Ophthalmology, Dorn Veterans Affairs Medical Center
V Al Pakalnis, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and South Carolina Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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