What Is CREST Syndrome? Everything to Know

Medically Reviewed By Stella Bard, MD
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CREST syndrome is a type of systemic scleroderma. It is a collagen vascular condition that results in thickening of the skin in certain areas. The condition is also called limited cutaneous systemic sclerosis. CREST syndrome is a milder form of systemic scleroderma that typically only affects the hands, feet, lower arms, lower legs, and face. In some cases, it can also affect the lungs and digestive system.

Symptoms of systemic scleroderma tend to appear between the ages of 30 and 50. CREST syndrome is a progressive condition, meaning that symptoms will gradually worsen over time.

Read on to learn more about CREST syndrome. This article includes information about causes and symptoms of the condition, as well as how to treat it.

What does CREST stand for?

There is a closeup of women holding hands.
Michela Ravasio/Stocksy United

“CREST” is an acronym that refers to five key features of the syndrome.

CREST stands for:

  • Calcinosis: This occurs when there are calcium deposits in the subcutaneous layer of the skin.
  • Raynaud’s phenomenon: This occurs due to extreme sensitivity to the cold, or as a result of stress.
  • Esophageal dysfunction: This refers to difficulties with swallowing due to scarring.
  • Sclerodactyly: This occurs when skin tightens around the fingers and toes.
  • Telangiectasia: This refers to red or discolored spots on the palms, hands, forearms, lips, and face.

What are the symptoms?

CREST syndrome often starts with circulation problems in the hands and feet. This is known as Raynaud’s phenomenon. Other symptoms include:

  • skin thickening
  • red or discolored spots
  • hard lumps under the skin
  • skin fibrosis, or the rapid growth of the skin’s connective tissue, which can cause:
    • tight, dry skin
    • pruritus or itchiness, which affects up to 43% of people with systemic scleroderma
    • accumulation of fluid
    • changes in skin pigmentation
  • difficulties swallowing
  • heartburn
  • hypothyroidism, which occurs in up to 15% of people with systemic scleroderma

Some people with CREST syndrome may develop pulmonary arterial hypertension.

In rare cases, a person with CREST syndrome may also experience scleroderma renal crisis or interstitial lung disease.

Calcinosis

With calcinosis, or calcinosis cutis, calcium accumulates below the outer layer of skin. If there is pressure on these areas of skin, you may experience:

  • severe pain
  • ulcerations
  • infection where calcium deposits protrude

Raynaud’s phenomenon

Raynaud’s phenomenon occurs when you experience blood vessel spasms, or vasospasms, in response to extreme cold or stress. This most often happens in the fingers, which turn white or discolor in response.

Fingers can also turn blue or discolor as vessels close up and cannot deliver oxygen. In turn, this can cause severe pain and ulcers.

Symptoms of Raynaud’s phenomenon typically last around 15–20 minutes, after which time blood can flow to the fingers again.

Raynaud’s phenomenon affects more than 95% of people with systemic scleroderma, compared with 15% of the general population without conditions affecting the connective tissue.

Learn more about Raynaud’s phenomenon.

Esophageal dysfunction

Esophageal dysfunction occurs when the lower esophagus becomes damaged or does not move as it should, causing difficulties with swallowing. This can also result in acid reflux and heartburn.

Visit our acid reflux and GERD hub for more information about acid reflux, heartburn, and related conditions.

Sclerodactyly

Sclerodactyly affects the skin over fingers and toes. Skin can become:

  • taut
  • shiny
  • thin

This can make it difficult for fingers and toes to bend. They may also become fixed in a flexed position, reducing movement and dexterity.

Telangiectasia

Telangiectasia refers to small blood vessels that are dilated and look like red or discolored lesions. These are typically visible on the:

  • face
  • tongue
  • lips
  • fingers
  • palms

How is CREST syndrome treated?

Treatments for CREST syndrome focus on managing symptoms and controlling any complications.

Medication your doctor prescribes may depend on which symptoms are present, but can include:

  • immunosuppressants to treat sclerodactyly, including:
    • methotrexate
    • mycophenolate mofetil
    • hydroxychloroquine
    • cyclophosphamide
  • calcium channel blockers to treat symptoms of Raynaud’s phenomenon, such as nifedipine (Procardia) and verapamil (Calan)
  • medication for high blood pressure to reduce symptoms of Raynaud’s disease, such as prazosin (Minipress) and methyldopa (Aldomet)
  • bisphosphonates to treat calcium deposits
  • prescription antacids to alleviate acid reflux and heartburn as a result of esophageal dysfunction
  • antihistamines and topical moisturizers to alleviate itchiness
  • ACE inhibitors for scleroderma renal crisis

Other treatments for symptoms of CREST syndrome include:

  • getting laser therapy to treat changes in pigmentation as a result of telangiectasia
  • participating in physical therapy to reduce the effects of sclerodactyly
  • eating smaller meals more frequently to reduce symptoms of esophageal dysfunction
  • wearing gloves, with or without silver fiber, to manage symptoms of Raynaud’s phenomenon
  • elevating the head of the bed to reduce symptoms of gastroesophageal reflux and heartburn

Learn more about treatments for different types of scleroderma.

What causes CREST syndrome?

As systemic scleroderma is an autoimmune condition, symptoms occur when the immune system mistakenly attacks the body. Excess fibrous tissue replaces healthy tissue, resulting in symptoms such as thickening of the skin and scarring of organs.

Medical professionals do not always know exactly what causes systemic scleroderma, which includes CREST syndrome.

However, there is a belief that triggers such as exposure to viruses or toxins may cause the condition to develop in people with a genetic disposition.

Possible environmental triggers that may contribute to the development of CREST syndrome include:

There are also links between drugs such as bleomycin and cocaine and the development of scleroderma-like conditions.

Are there links between CREST syndrome and the COVID-19 vaccine?

There is no evidence that suggests that the vaccine for COVID-19 can cause CREST syndrome or other autoimmune conditions.

When should I contact a doctor?

It is important to contact your doctor as soon as you experience symptoms of CREST syndrome. Early diagnosis is crucial for managing the condition and its effects on internal organs.

How is CREST syndrome diagnosed?

Your doctor will assess your medical history and carry out a physical examination. If they suspect that you have a type of systemic scleroderma, such as CREST syndrome, they can arrange for tests.

Tests and examinations that can help with diagnosing CREST syndrome include:

  • tests to check for antinuclear antibodies, which are present in more than 90% of systemic sclerosis cases
  • tests to check for specific autoantibodies that are present in more than 70% of cases, including:
    • anti-centromere
    • anti-SCL70
    • anti-RNA polymerase III
  • evaluation of skin thickness using the modified Rodnan skin score, which rates uninvolved skin as 0 and severe skin thickening as 3
  • nailfold capillary exam, which helps distinguish between Raynaud’s phenomenon and secondary Raynaud’s disease
    • Also called nailfold capillary dermatoscopy, this exam involves using a magnification device such as a magnifying glass to observe the density of nailfold capillaries.
  • complete blood count to test for anemia, which can indicate scleroderma renal crisis
  • X-rays to identify calcinosis
  • tests and procedures for esophageal dysfunction, including:

Are there any complications of CREST syndrome?

Possible complications of CREST syndrome include:

  • damage to organs as a result of fibrosis
  • malnutrition due to gastrointestinal symptoms
  • renal damage from scleroderma renal crisis

Contact your doctor as soon as you experience symptoms of CREST syndrome. Early monitoring of the condition can help to reduce the risks of complications.

Summary

CREST syndrome, or limited cutaneous systemic sclerosis, is a type of systemic scleroderma characterizable by thickening of the skin. It typically affects the hands, feet, lower arms, lower legs, and face.

You can manage symptoms of CREST syndrome with prescription medication. You can also take steps to alleviate symptoms, including wearing gloves, eating smaller meals more frequently, and elevating the head of your bed.

Contact your doctor if you have concerns about CREST syndrome. Early detection can help with managing and monitoring the condition.

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Medical Reviewer: Stella Bard, MD
Last Review Date: 2022 Jun 20
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THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
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  4. Liem, S. I. E., et al. (2022). The effect of silver fibre gloves on Raynaud's phenomenon in patients with systemic sclerosis: A double-blind randomized cross-over trial. https://academic.oup.com/rheumatology/advance-article/doi/10.1093/rheumatology/keac243/6571140?login=false
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