Pamintuan, Maren P.

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Systemic Sclerosis (Scleroderma)

Systemic Disease with Oral Manifestation

Etiology
The causes are not fully known. There is some evidence that genetic and
environmental factors play a role in the genesis of scleroderma. Silica and
certain organic solvents are recognized as risk factors of occurrence of
systemic scleroderma. The result is an activation of the immune system,
causing blood vessel damage and injury to tissues that result in scar tissue
formation and the accumulation of excess collagen.
Genetic factors play at least a limited role. According to three US cohorts,
the prevalence of the disease was 13 times higher in first-degree relatives
of scleroderma patients than in the general population. OX40L gene
polymorphism correlates with systemic scleroderma. IRF5 gene was
found to correlate with systemic scleroderma as well as with the
occurrence of interstitial lung disease during scleroderma

Sign and Symptoms

Depend on which parts of your body are affected:

Skin. Nearly everyone who has scleroderma experiences a hardening and

tightening of patches of skin. These patches may be shaped like ovals or
straight lines, or cover wide areas of the trunk and limbs. The number,
location and size of the patches vary by type of scleroderma. Skin can
appear shiny because it's so tight, and movement of the affected area may
be restricted.

Fingers or toes. One of the earliest signs of systemic scleroderma is

Raynaud's disease, which causes the small blood vessels in your fingers
and toes to contract in response to cold temperatures or emotional
distress. When this happens, your fingers or toes may turn blue or feel
painful or numb. Raynaud's disease also occurs in people who don't have
scleroderma.

Digestive system. Scleroderma can cause a variety of digestive

symptoms, depending on which part of the digestive tract is affected. If
the esophagus is affected, you might have heartburn or difficulty
swallowing. If the intestines are affected, you might have cramps,
bloating, diarrhea or constipation. Some people who have scleroderma
may also have problems absorbing nutrients if their intestinal muscles
aren't properly moving food through the intestines.

Heart, lungs or kidneys. Scleroderma can affect the function of the heart,

lungs or kidneys to varying degrees. These problems, if left untreated, can
become life-threatening.

Diagnosis
Scleroderma can take so many forms and affect so many different areas
of the body, it can be difficult to diagnose.

After a thorough physical exam, may suggest blood tests to check for
elevated levels of certain antibodies produced by the immune system. He
or she may also remove a small sample of your affected skin so that it can
be examined in the laboratory. It suggest other blood tests and imaging or
organ-function tests to help determine whether your digestive system,
heart or lungs are affected.

Scleroderma is a clinical diagnosis that takes into account all factors,

including the physical examination and all symptoms. A blood test alone
cannot diagnose scleroderma. Depending on the clinical situation,
additional tests may be done, such as:

 Pulmonary function tests or breathing tests to measure how well the

lungs are working.
 CT chest scan may also be ordered to evaluate the extent of lung
involvement.
 Electrocardiogram (EKG or ECG) to see if there are changes in the
heart muscle tissue due to scleroderma. An EKG/ECG records the
electrical activity of the heart, shows abnormal rhythms and detects
any damage.
 Echocardiogram to look at the structure and function of the heart. It
uses sound waves to take pictures of the heart and valves.
 X-rays or special imaging to show any changes in the bones or soft
tissues caused by scleroderma. It uses a small amount of radiation to
take pictures of internal tissues, bones and organs.
 Motility studies to assess for gastrointestinal dysmotility.

Differential Diagnosis
 Other conditions may cause changes of the skin clinically close to those
observed during scleroderma.

 Scleredema (scleredema diabeticorum and scleredema of Buschke) is

characterized by skin thickening, affecting the neck, upper back,
shoulders, and trunk. Raynaud phenomenon and internal organ
involvement are absent.
 Eosinophilic fasciitis, often occurring following an unusual effort and
characterized by edematous diffuse infiltration of the forearms and
legs. It leads to the adherence of the skin to underlying fascia, usually
sparing the hands and feet. Raynaud phenomenon is absent.
 Eosinophilia-myalgia syndrome, which is a form of eosinophilia that
is caused by L-tryptophan.Graft-versus-host disease, an autoimmune
pathology that occurs as a result of bone marrow transplants in which
the immune cells from the transplanted bone marrow attack the host's
body.
 Nephrogenic systemic fibrosis, which is usually caused by kidney
failure. Skin changes may be indistinguishable from scleroderma, but
Raynaud phenomenon is typically absent. May have internal organ
fibrosis.

Treatment
Scleroderma fade away on their own in two to five years, the type of that
affects internal organs usually worsens with time.

Medications
There is no medication that can cure or stop the overproduction of
collagen that is characteristic of scleroderma. But a variety of
medications can help control scleroderma symptoms and prevent
complications. For example, your doctor may recommend medications to:

 Treat or slow skin changes. Steroid creams or pills may help reduce

swelling and joint pain, loosen stiff skin, and slow the development of
new skin changes.
 Dilate blood vessels. Blood pressure medications that dilate blood
vessels may help prevent lung and kidney problems and may help
treat Raynaud's disease.
 Suppress the immune system. Drugs that suppress the immune
system, such as those taken after organ transplants, may help reduce
scleroderma symptoms.
 Reduce digestive symptoms. Pills to reduce stomach acid can help
relieve heartburn. Antibiotics and medications that help move food
through the intestines may help reduce bloating, diarrhea and
constipation.
 Prevent infections. Antibiotic ointment, cleaning and protection from
the cold may help prevent infection of fingertip ulcers caused by
Raynaud's disease. Regular influenza and pneumonia vaccinations
can help protect lungs that have been damaged by scleroderma.
 Relieve pain. If over-the-counter pain relievers don't help enough, you
can ask your doctor to prescribe stronger medications.

Therapies
Physical or occupational therapists can help:

 Manage pain
 Improve your strength and mobility
 Maintain independence with daily tasks

Surgery
Used as a last resort, surgical options for scleroderma complications may
include:

 Amputation. If finger sores caused by severe Raynaud's disease have

progressed to the point that the fingertip tissue begins to die,
amputation may be necessary.
 Lung transplants. People who have developed severe lung problems
may be candidates for lung transplants.

Management
 Stay active. Exercise keeps your body flexible, improves circulation
and relieves stiffness. Range-of-motion exercises can help keep your
skin and joints flexible.
 Protect your skin. Take good care of dry or stiff skin by using lotion
and sunscreen regularly. Avoid hot baths and showers and exposure
to strong soaps and household chemicals, which can irritate and
further dry out your skin.
 Don't smoke. Nicotine causes blood vessels to contract, making
Raynaud's disease worse. Smoking can also cause permanent
narrowing of your blood vessels, and cause or exacerbate lung
problems. Quitting smoking is difficult — ask your doctor for help.
 Manage heartburn. Avoid foods that give you heartburn or gas. Also
avoid late-night meals. Elevate the head of your bed to keep stomach
acid from backing up into your esophagus as you sleep. Antacids may
help relieve symptoms.
 Protect yourself from the cold. Wear warm mittens for protection
anytime your hands are exposed to cold — even when you reach into
a freezer. When you're outside in the cold, cover your face and head
and wear layers of warm clothing.

Coping and support

As is true with other chronic diseases, living with scleroderma can place
you on a roller coaster of emotions.

 Maintain normal daily activities as best you can.

 Pace yourself and be sure to get the rest that you need.
 Stay connected with friends and family.
 Continue to pursue hobbies that you enjoy and are able to do.

Dental Consideration
 Inability to open mouth
 Oral Dryness Increase water intake, ice chips
 Dry mouth OTC products
- Saliva gel/spray, mouth rinses, sugarless candy/gum
 Pilocarpine and Cevimeline (muscarinic agonists)
 Oral Candida Infection

Radiographic evidence of Progressive Scleroderma

 Thickening of the lamina dura of teeth in dental xrays is typically
diagnostic of progressive scleroderma.
CREST syndrome
 Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility,
Sclerodactyly, and Telangiectasia
 Shutdown of microvascular circulation and contribution to
periodontal disease
 Decreased healing

Significant associated findings that you have noted on

the case
 characterized by dense collagen deposition within the tissues and
ranges from localized to systemic disease.
 females are more commonly affected.
 oral findings are variable, including changes to the lips and mouth.
 lips appear pursed, and opening of the mouth may be limited.
 Xerostomia is common; the tongue appears smooth, as do the palatal
rugae.
 panoramic radiographs, mandibular resorption may be noted.

Risk factors
 being Native American
 being African-American
 being female
 using certain chemotherapy drugs such as Bleomycin
 being exposed to silica dust and organic solvents

 Odonwodo A, Badri T, Hariz A.(2020). Scleroderma. StatPearls.

 Heidi L. Gaddey, MD.(2017).Oral manifestations of systemic

disease.Published with permission of the Academy of General
Dentistry

 https://www.aad.org/public/diseases/a-z/scleroderma-symptoms

 https://www.scleroderma.org/site/SPageNavigator/patients_whatis.ht
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