CREST Syndrome


Definition

Hardening of the skin in a particular region is termed as CREST syndrome. This is a subtype of scleroderma and though it affects regionally, therefore it can also called as limited scleroderma.

The usual sites of development of the CREST Syndrome, include the lower part of the hands and legs. CREST Syndrome also occurs in the face and throat. Internal organs like elementary cannel and in severe condition, heart or lungs can also affect.

The illustration of CREST can be described as

C – Deposition of calcium in epidermis layer and subcutaneous tissue of the skin ( calcinosis)


R – failure of muscle control (Raynaud’s phenomenon) in the affected skin and tissues and even organ like esophagus.

E –  Esophageal muscles become weakened and swallowing difficulty arises due to dysmotility.

S –  a thinning malformation of the bones of the fingers and the skin becomes thicken (Sclerodactyly).

T – Enlargement of the blood vessels in the affected part of the body (telangiectasias), due to this red spots occur on the face and other affected areas. (1,2,3)


Crest Syndrome definition


Symptoms

The following symptoms are noticeable for CREST syndrome:

Tight hardened skin

Calcium deposition in the skin tissue often leads to tightening and hardened skin tissues and this is often caused pain and irritation of the skin. Excessive calcium deposition can also cause breaking of the skin surface. Due to this patient experience difficulty in bending fingers or mouth opening. The tightening of the skin may increase the glossiness of the affected surface area.

CREST syndrome picture

Raynaud’s phenomenon

The blood vessels present in the affected fingers and toes get spasm due to cold weather or mental stress. This can also lead to blocking of the blood circulation and skin become white and then turns to  blue, the temperature of the skin becomes lower down and numbness raises. Further improvement of the blood flow can cause throbbing or tingling sensation along with reddish skin.

Crest syndrome symptoms raynauds

Red spot marks on the skin

The swelling of the small blood vessels beneath the skin surface can cause painless red spot marks on the affected skin surface.

Crest syndrome systemic sclerosis

Lumps beneath the skin

In rare cases, calcium deposition beneath the skin appears as a lump (telangiectasias) and mainly found in the fingers, elbows or knee. These lumps may be infected and tenderness also develop.

Difficulty in swallowing

In CREST syndrome, people often face swallowing difficulties, due to their problem in the esophagus. The loss of control over the esophageal muscles at the upper and lower end can cause difficulty in swallowing and let back flow the acidic content of the stomach into the esophagus. This leads to heartburn, irritation and scarring of the esophagus. (1,2,3,4)

Classification

In medical science, depending upon the sign and symptoms, CREST syndrome is further divided into two forms:

“Pure”CREST — it means when the diagnostic reports reveled that patient have two or more than two symptoms of CREST and not associated with diffuse scleroderma or other autoimmune disease.
Plus CREST – It means when the patient has CREST syndrome along with another form of scleroderma, including diffuse scleroderma or other types of autoimmune disease symptoms.
In diffuse scleroderma the involved skin is not localized in a particular area and the involvement of the internal organs like the digestive system, heart, lungs and kidney. (2,4)

Causes of CREST Syndrome

It assumed that CREST syndrome is developed due to an autoimmune disorder, means the immune system of one’s body can act against own body. This assumption comes from the pathophysiology involved in developing the CREST Syndrome. In CREST syndrome, the collagen synthesis in increased due to influence of the immune system. Collagen is one of the chief constituents of the immune system of the connective tissue. The excess amount of collagen retain in the cutaneous and subcutaneous layer of the skin and internal organs, weakening their function and cause different deformities.

It is presumed that Anticentromere Antibodies (ACA)  are the role in the development of the CREST syndrome, though it is not found in every involved ethnic group (Afro-American, south-African, Indian, and Thai people) in further studies.  (1,2)

Risk factors

Females are more susceptible than males.
Incidence rate of CREST syndrome is higher in blacked skin people, in contrast with the whites.
Family history has the presence of an autoimmune disease, including rheumatoid arthritis, lupus, or Hashimoto’s disease, the chance of the development of CREST syndrome is more due to genetic involvement.
Certain toxic chemicals like benzene, polyvinyl chloride, trichloroethylene and silica may elicit CREST syndrome due to genetic disposition to the ailment.(1)

Complications

Apart from the symptomatic approach, untreated condition may contribute further complications, which include:

If the CREST syndrome extends into the gut wall, often it provides constipation, flatulence, loose motion, malnutrition and unexplained weight loss.
Regional blood circulation can totally obstruct in case of a severe Raynaud’s phenomenon and ulcer may develop. Usually fingers and toes often get affected and delayed healing leads to increase the chance of gangrene of fingers or toes due to narrowed blood vessels.
Stiffness of the lung tissue lead to a lower functioning capability of the lungs and  can cause an increase work load of the heart and heart muscle becomes weakened.
Cardiac problem like arrhythmias and myocarditis gradually develop due to progression of the CREST syndrome.
Severe contraction of the facial skin leads to difficulty in opening and widening  mouth and it may create difficulty in brushing of the teeth. Due to esophageal impairment can cause  acid reflux  and that able to obliterate tooth enamel, and alteration of the gum muscles may lead to loosen tooth hold.
In severe CREST syndrome patient also complain about dry eyes and mouth.  (1)

Diagnosis

The CREST syndrome diagnosis is based on symptomatic examination and medical history analysis. In addition following tests are also conducted for determination of the underlying cause and severity judgment:

Blood test

Analysis of the involve antibodies is assessed through a blood test, though this is not provided confirmatory result, as every individual has not contain the suspected identified antibodies.

Biopsy of the skin sample

Doctors can take a small skin sample of the affected area, for biopsy test. This may not helpful to determine the CREST symptom, but it confirms that malignancy is not involved in the alteration of the skin structure.

Heart, lung and gastrointestinal tests

The different test related to heart, lungs and gastrointestinal tract also conduct to estimate the internal organ involvement and severity of the CREST syndrome.  (1)

CREST Syndrome Treatment

CREST syndrome is incurable condition, the available treatment options help to reduce the symptomatic manifestations and restrict probable complications.
Pharmaco-therapies

Antibiotics

Topical antibiotics are prescribed to apply the affected area, if the superficial skin surface become infected.

Antacids

For symptomatic relieve like heartburn can be minimized by neutralizing the acid content of the upper part of the stomach and esophagus and antacids are acting against the excessive acid content and higher the pH.

Antihypertensive drugs

Narrowing of the blood vessels often causes hypertension and increase the load in cardiac muscle, to control this antihypertensive drugs are often prescribed.

Immunosuppressive medications

The medicines under this category helps to prevent the development of the involved antibodies and also limit the collagen retention in the skin tissues and internal organs.

Rehabilitation Therapy

Physiotherapy is applied to control the progression of the stiffness of the joints and helps to maintain the flexibility of the affected joint. Physiotherapy also beneficial to reduction of the pain.
Occupational therapy

In progressive CREST syndrome may hamper daily activity, like brushing teeth, ingestion of food, occupational therapy can help to reduce the daily life problem and improve the quality of life of the patient.
Surgical intervention

In excessive calcium deposition, or rectification of the nuisance of the red spots on the skin (Laser surgery)and finger and toe gangrene  need surgical intervention (amputation). (1)


References

  • Limited scleroderma (CREST syndrome), (2014); Mayo Clinic Staff; Retrieve from: http://www.mayoclinic.org/diseases-conditions/crest-syndrome/basics/treatment/con-20031524
  • Systemic Sclerosis (Scleroderma): CREST Syndrome; Sclero.Org;  Retrieve from: http://www.sclero.org/scleroderma/types/systemic/limited/crest/a-to-z.html
  • CREST Syndrome and Scleroderma, (2014); WebMD Medical Reference; Retrieve From: http://www.webmd.com/skin-problems-and-treatments/crest-syndrome
  • Types of Scleroderma; Johns Hopkins Scleroderma Center; Retrieve from: http://www.hopkinsscleroderma.org/scleroderma/types-scleroderma/

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