eMedicine Specialties > Dermatology > Diseases of the Dermis

Pseudoxanthoma Elasticum: Follow-up

Author: L Frank Glass, MD, Chief of Dermatopathology, Associate Professor, Departments of Internal Medicine and Pathology, University of South Florida College of Medicine
Coauthor(s): M Michelle (Shellie) Marks, MD, Staff Physician, Department of Dermatology, University of Alabama at Birmingham; Daniel F Smith, MD, Consulting Staff, Bressinck, Gibson, Parker, Fangster, Smith, and Bruyneel Dermatology; Neil Alan Fenske, MD, Chairman, Department of Dermatology and Cutaneous Surgery, Professor, Department of Dermatology and Cutaneous Surgery, Department of Pathology and Cell Biology, Department of Oncologic Sciences, University of South Florida College of Medicine; Sujatha Tadicherla, MD, Resident, Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine
Contributor Information and Disclosures

Updated: Nov 21, 2008

Follow-up

Further Outpatient Care

  • Regular fecal occult blood testing and CBC count should be performed every 6 months to 1 year to monitor for gastrointestinal hemorrhaging.
  • An ophthalmologic examination should be performed on pseudoxanthoma elasticum (PXE) patients at least once a year to detect early retinopathy, angioid streaks, or retinal hemorrhage.
  • Regular physical examinations, with particular attention to the cardiovascular system, should be performed to detect mitral valve insufficiency, coronary artery disease, or peripheral vascular compromise.

Complications

  • Pseudoxanthoma elasticum (PXE) ocular involvement with retinal hemorrhages can lead to the progressive loss of central vision. Peripheral vision is always spared.
  • Involvement of the elastic media and intima of the arteries can lead to claudication, hypertension, angina, myocardial infarction, and gastrointestinal or cerebral hemorrhage.
  • Cerebral and gastrointestinal hemorrhage or coronary occlusion, although uncommon, may be fatal.

Prognosis

  • The prognosis of pseudoxanthoma elasticum (PXE) largely depends on the extent of extracutaneous organ involvement. Patients typically have a normal life span, but acute gastrointestinal hemorrhage, myocardial infarction, or cerebral hemorrhage may be fatal.

Miscellaneous

Special Concerns

  • Pregnancy is not contraindicated in pseudoxanthoma elasticum (PXE) patients; however, the risk of miscarriage is increased in the first trimester.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.



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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Further Reading

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Keywords

pseudoxanthoma elasticum, PXE, Grönbald-Strandberg syndrome, 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

L Frank Glass, MD, Chief of Dermatopathology, Associate Professor, Departments of Internal Medicine and Pathology, University of South Florida College of Medicine
L Frank Glass, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology
Disclosure: Nothing to disclose.

Coauthor(s)

M Michelle (Shellie) Marks, MD, Staff Physician, Department of Dermatology, University of Alabama at Birmingham
Disclosure: Nothing to disclose.

Daniel F Smith, MD, Consulting Staff, Bressinck, Gibson, Parker, Fangster, Smith, and Bruyneel Dermatology
Daniel F Smith, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, and Arkansas Medical Society
Disclosure: Nothing to disclose.

Neil Alan Fenske, MD, Chairman, Department of Dermatology and Cutaneous Surgery, Professor, Department of Dermatology and Cutaneous Surgery, Department of Pathology and Cell Biology, Department of Oncologic Sciences, University of South Florida College of Medicine
Disclosure: Dermik Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching; Graceway Pharmaceuticals Honoraria Speaking and teaching; Abbott Honoraria Speaking and teaching; Warner Chilcott Honoraria Speaking and teaching

Sujatha Tadicherla, MD, Resident, Department of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine
Disclosure: Nothing to disclose.

Medical Editor

Mark G Lebwohl, MD, Chairman, Department of Dermatology, Mount Sinai School of Medicine
Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Abbott Laboratories Honoraria Consulting; Actelion Honoraria Consulting; Amgen Honoraria Consulting; Astellas Honoraria Consulting; Centocor Honoraria Consulting; DermiPsor Honoraria Consulting; Galderma  Consulting; Genentech Honoraria Consulting; Helix BioMedix Honoraria Consulting; Medicis Honoraria Investigator

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Christen M Mowad, MD, Associate Professor, Department of Dermatology, Geisinger Medical Center
Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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