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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Dermatology · Pseudoxanthoma Elasticum

How PXE Affects Your Body: Symptoms and Standard Treatments

At a Glance

Pseudoxanthoma elasticum (PXE) is a systemic disorder that causes mineral buildup in the body's elastic fibers. It primarily affects the skin, eyes, cardiovascular system, and GI tract. Key treatments focus on monitoring and managing complications like bleeding eye vessels and stiffened arteries.

Pseudoxanthoma elasticum (PXE) is a systemic disorder that affects several major organ systems [1]. Because minerals build up in the body’s elastic fibers, the effects are most visible and impactful in the skin, the eyes, the cardiovascular system, and the gastrointestinal tract [2].

The Skin: Early Warning Signs

For many, the first signs of PXE appear on the skin, particularly in areas where the skin folds, like the neck, underarms, or groin [3].

  • Appearance: Small, yellowish bumps (papules) may cluster together, giving the skin a pebbled texture often compared to “plucked chicken skin” [4]. Over time, these bumps may merge, leading to areas of loose or sagging skin (often called redundant skin) [5].
  • Dermoscopy: When a dermatologist looks at these lesions through a specialized magnifying tool, they see a distinct net-like pattern of yellow-white clods on a purple or red background, which helps confirm it is true PXE [6].

The Eyes: Protecting Your Vision

The most serious complications of PXE often involve the eyes, specifically a layer called the Bruch’s membrane [7].

  • Angioid Streaks: These are cracks that develop in the calcified Bruch’s membrane, looking like jagged, reddish-brown lines radiating from the optic nerve [8].
  • CNV (Choroidal Neovascularization): These cracks can allow abnormal, fragile blood vessels to grow under the retina. If these vessels leak or bleed, they cause progressive vision loss [9][10].
  • Standard of Care: The treatment for leaking vessels is Anti-VEGF injections. These medications are injected into the eye to shrink the abnormal vessels and prevent vision loss [11].
  • Daily Safety & Monitoring: You should use an Amsler grid daily—a simple square grid of straight lines. If the lines appear wavy, it may indicate a new bleed [11]. Because the eye vessels are fragile, you should avoid high-impact contact sports, heavy lifting, or activities with a risk of head trauma, as blunt force can trigger sudden bleeding.

The Gastrointestinal (GI) Tract: Bleeding Risks

PXE can cause the blood vessels in your stomach and intestines to become calcified and fragile, significantly increasing the risk of gastrointestinal bleeding [2][12].

  • Warning Signs: Watch for symptoms like dark, tarry stools or vomiting blood. If you experience these, seek emergency medical care immediately.
  • Medication Safety: To minimize bleeding risks, you should strictly avoid over-the-counter pain relievers that thin the blood, like ibuprofen, naproxen (NSAIDs), and aspirin, unless specifically prescribed by your doctor. Use safer alternatives like acetaminophen for everyday pain.

The Cardiovascular System: Managing “Stiff” Arteries

Because PXE causes the walls of the arteries to mineralize, it can affect how blood flows throughout your body [13].

  • Arterial Calcification: This leads to premature arterial stiffness and can cause claudication (leg pain while walking) or premature heart disease [14][15].
  • Essential Screenings: Doctors will monitor your heart and blood vessels using tools like the ABI (Ankle-Brachial Index) to check for blockages in your legs, as well as specialized stress tests and ultrasound imaging [14][16].
  • Diet and Supplements: Doctors often advise patients to be cautious with calcium and Vitamin D supplements, as excessive, concentrated doses might speed up the calcification process [17]. However, normal dietary calcium from food (like dairy or leafy greens) is completely safe and necessary for your bone health. Do not restrict your natural diet without speaking to your doctor.

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Common questions in this guide

What are the first signs of PXE on the skin?
The earliest skin signs are typically small, yellowish bumps that form in skin folds like the neck, underarms, or groin. Over time, these bumps can merge and cause the skin to become loose or sagging.
How does PXE affect my eyes and vision?
PXE can cause cracks in the back of the eye called angioid streaks. These cracks allow abnormal, fragile blood vessels to grow and leak, which can lead to progressive vision loss if not caught and treated early.
What is the standard treatment for PXE eye complications?
If abnormal blood vessels begin leaking in the eye, the standard treatment is anti-VEGF injections. These medications help shrink the fragile blood vessels and protect your vision from further damage.
Can I take NSAIDs or aspirin if I have PXE?
You should generally avoid over-the-counter pain relievers that thin the blood, such as ibuprofen, naproxen, and aspirin. Because PXE makes gastrointestinal blood vessels fragile, these medications can significantly increase your risk of dangerous internal bleeding.
Why do my legs hurt when I walk if I have PXE?
PXE causes calcium buildup in your arteries, making them stiff and narrowing blood flow. This reduced circulation can cause claudication, which is a cramping pain in the legs that occurs during physical activity like walking.
Should I avoid calcium in my diet if I have PXE?
Normal dietary calcium from foods like dairy and leafy greens is completely safe and necessary for bone health. However, you should be cautious with calcium and Vitamin D supplements, as concentrated doses might speed up the calcification process in your arteries.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is my current risk of developing Choroidal Neovascularization (CNV) based on my latest eye exam?
  2. 2.How often should I have an Ankle-Brachial Index (ABI) test to monitor my arterial health?
  3. 3.Are there specific cardiovascular stress tests you recommend given the risk of premature arterial calcification?
  4. 4.Are my current over-the-counter pain medications safe for my gastrointestinal tract?
  5. 5.How do you coordinate with my dermatologist, cardiologist, and ophthalmologist to manage my PXE holistically?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (17)
  1. 1

    Cutaneous and oral manifestations of pseudoxanthoma elasticum: clinicopathological features of an uncommon disorder.

    Teixeira LR, Chahud F, Simão JCL, et al.

    Clinical and experimental dermatology 2021; (46(4)):745-748 doi:10.1111/ced.14549.

    PMID: 33378100
  2. 2

    ABCC6 and Pseudoxanthoma Elasticum: The Face of a Rare Disease from Genetics to Advocacy.

    Moitra K, Garcia S, Jaldin M, et al.

    International journal of molecular sciences 2017; (18(7)) doi:10.3390/ijms18071488.

    PMID: 28696355
  3. 3

    Pseudoxanthoma Elasticum With Comedones in a 12-Year-Old Female Patient: A Case Report.

    Almutairi R, Al-Sabah H

    Cureus 2023; (15(10)):e47041 doi:10.7759/cureus.47041.

    PMID: 38022106
  4. 4

    Threads of Elasticity: A Single Variant Journey Through Pseudoxanthoma Elasticum's Clinical Maze.

    Nagarajan DR, Mani Jacob D, Kakade S

    Cureus 2025; (17(8)):e89237 doi:10.7759/cureus.89237.

    PMID: 40901220
  5. 5

    Correlation of systemic involvement and presence of pathological skin calcification assessed by ex vivo nonlinear microscopy in Pseudoxanthoma elasticum.

    Fésűs L, Kiss N, Farkas K, et al.

    Archives of dermatological research 2023; (315(7)):1897-1908 doi:10.1007/s00403-023-02557-x.

    PMID: 36847829
  6. 6

    Dermoscopic features of pseudoxanthoma elasticum.

    Kawashima S, Togawa Y, Miyachi H, Matsue H

    Clinical and experimental dermatology 2018; (43(2)):175-179 doi:10.1111/ced.13308.

    PMID: 29271496
  7. 7

    Bruch's Membrane Calcification in Pseudoxanthoma Elasticum: Comparing Histopathology and Clinical Imaging.

    Risseeuw S, Pilgrim MG, Bertazzo S, et al.

    Ophthalmology science 2024; (4(2)):100416 doi:10.1016/j.xops.2023.100416.

    PMID: 38170125
  8. 8

    Coquille d'oeuf in young patients affected with Pseudoxantoma elasticum.

    Murro V, Mucciolo DP, Giorgio D, et al.

    Ophthalmic genetics 2019; (40(3)):242-246 doi:10.1080/13816810.2019.1627466.

    PMID: 31269855
  9. 9

    The Extent of Angioid Streaks Correlates With Macular Degeneration in Pseudoxanthoma Elasticum.

    Risseeuw S, Ossewaarde-van Norel J, van Buchem C, et al.

    American journal of ophthalmology 2020; (220()):82-90 doi:10.1016/j.ajo.2020.07.022.

    PMID: 32702361
  10. 10

    Aflibercept for choroidal neovascularizations secondary to pseudoxanthoma elasticum: a prospective study.

    Gliem M, Birtel J, Herrmann P, et al.

    Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie 2020; (258(2)):311-318 doi:10.1007/s00417-019-04551-4.

    PMID: 31863395
  11. 11

    Angioid Streaks in Pseudoxanthoma Elasticum.

    Mandura RA, Radi RE

    Cureus 2021; (13(6)):e15720 doi:10.7759/cureus.15720.

    PMID: 34277296
  12. 12

    Mutation spectrum in the ABCC6 gene and genotype-phenotype correlations in a French cohort with pseudoxanthoma elasticum.

    Legrand A, Cornez L, Samkari W, et al.

    Genetics in medicine : official journal of the American College of Medical Genetics 2017; (19(8)):909-917 doi:10.1038/gim.2016.213.

    PMID: 28102862
  13. 13

    Pseudoxanthoma elasticum: An unique entity in the coronary surgery.

    Aljasem H, Ali-Ghosh H, Kiran T, Ohri S

    Perfusion 2025; (40(5)):1281-1283 doi:10.1177/02676591241288180.

    PMID: 39317383
  14. 14

    Arterial stiffness is related to a higher risk of cardiovascular events in patients with pseudoxanthoma elasticum (PXE).

    Haverkamp M, de Jong PA, Visseren FL, Spiering W

    Vascular medicine (London, England) 2026; (31(1)):19-27 doi:10.1177/1358863X251394284.

    PMID: 41566718
  15. 15

    Increased Intracranial Arterial Pulsatility and Microvascular Brain Damage in Pseudoxanthoma Elasticum.

    Bartstra JW, van den Beukel T, Kranenburg G, et al.

    AJNR. American journal of neuroradiology 2024; (45(4)):386-392 doi:10.3174/ajnr.A8212.

    PMID: 38548304
  16. 16

    Successful revascularization of advanced coronary artery disease associated with pseudoxanthoma elasticum.

    Anzai F, Kunii H, Kanno Y, et al.

    Journal of cardiology cases 2017; (16(4)):101-104 doi:10.1016/j.jccase.2017.05.001.

    PMID: 30279808
  17. 17

    Choroidal neovascularization secondary to angioid streaks in a patient with pseudoxanthoma elasticum: case report.

    Beuran DI, Constantin C, Șerban RC, et al.

    Romanian journal of ophthalmology 2024; (68(4)):470-475 doi:10.22336/rjo.2024.85.

    PMID: 39936052

This page provides educational information about PXE symptoms and standard treatments. Always consult your medical team before changing medications, starting new supplements, or treating new symptoms.

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