Scleroderma
{ A connective tissue
disease affecting the entire body }
Oral and TMJ Manifestations are Common
Scleroderma
is a rather rare connective tissue disease that's
characterized by inflammation and excessive fibrosis (the
formation of fibrous connective tissue as a reparative or
reactive process). This disease was formerly known as,
"skin-bound" or "hide-bound" disease.
Both of these terms
accurately describe the ultimate effects of thickening of
the skin due to swelling and thickening of the underlying
connective tissue. Scleroderma is not
contagious
and only inherited in rare occasions. The actual cause is
unknown, but vascular, endocrine and autoimmune factors
have been suspected. Despite the actual cause, there is
one known common course of the disease: an exaggerated
production of collagen, the most abundant connective
tissue fiber in the body. About 19 persons per million
are afflicted with scleroderma each year. Women are
effected about three times more than men and the average
age of onset of this disease is 40 years of age.
Unfortunately, scleroderma is a chronic condition may
have a fatal outcome within a few years.
There are two general types: localized and systemic
(generalized). The localized form is usually not as
severe as the generalized form.
LOCALIZED
SCLERODERMA.
The localized type of scleroderma affects mainly the
skin, but bones and muscles are also possible targets. It
is not a severe as the generalized type, but it does lead
to a gradual decrease in joint mobility, the hands and
TMJs being most affected. Also, calcifications form under
the skin and the walls of arteries become rigid and
thickened.
Oral,
Facial and TMJ Involvement. Generalized oral manifestations of
localized scleroderma include fibrosis of the tongue and
soft palate, thinning of the lips, esophageal
dysfunction, and often neuralgia-like pain in the
trigeminal nerve may develop. When the facial muscles are
involved, a progressive limitation of mouth-opening
develops (microstomia) due to the decreasing elasticity
of the skin and narrowing of the lips. The nose may
become pointed and seem to be covered with shiny skin
("mouse facies"). Over the cheeks, the skin may
draw tightly with the color appearing yellow-white or
pale. The face becomes taught and hard as the disease
progresses, resulting in a condition termed
"mast-like" facies. Mobility of the eyelids and
cheeks becomes decreased.
The tongue may become smooth and a condition
termed frenulum sclerosis, or thickening of the
attachment of the tongue to the floor of the mouth
develops. This further complicates routine cleaning of
the mouth and teeth.
When the TMJ is involved, pain, swelling and joint noise
occur. Reduction of jaw movement may ultimately lead to
the formation of adhesions and inflammation in the TMJs,
destruction of the mandibular condyle and coronoid
process.
In addition, TMJs affected by scleroderma destroy the
normal occlusion (bite) which leads to the
development of frontal occlusion, or, biting primarily on
the front teeth only. This change in the occlusion is
caused by deformation of the surfaces of the mandibular
condyle and articular eminence, the body of the mandible,
and even the corner or angle of the mandible. This type
of change in occlusion is also common in such destructive
diseases as rheumatoid arthritis and polyarthritis.
SYSTEMIC
(GENERALIZED) SCLERODERMA. Generally, those effected with systemic
scleroderma complain of being tired, depressed and suffer
with headaches. Acrocyanosis (a circulation disorder in
which the hands, and at times, the feet, are persistently
cold, blue and sweaty) is also a sign. This phenomenon,
termed Raynaud's syndrome (bluing and cold feeling of
extremities due to vascular spasms), ultimately develops
in about 70-90% of those afflicted with this disease.
Laboratory tests reveal an elevated serum glutamic oxalic
transaminase, lactic dehydrogenase, and aldolase because
of muscle involvement. Altered antibodies produce a
positive latex fixation test in about 40% of scleroderma
patients. In addition, there is an elevation in the
erythrocyte sedimentation rate, rheumatoid factor, and an
increase in urine excretion of proline. These laboratory
tests are positive for both localized and generalized
scleroderma.
There are two sub-types of systemic scleroderma: limited
and diffuse. Limited is also termed the CREST
syndrome.
This acronym stands for the following combination of
symptoms: Calcinosis (deposition of calcium), Raynaud's
syndrome, Esophageal dysfunction, Sclerodactyly (skin of
fingers becomes stiff, smooth, shiny) , and
Telangiectasia (bleeding from superficial dilated
capillaries). CREST syndrome usually has a slow
beginning, with the first symptoms appearing 10 to 20
years before all the symptoms manifest. Usually, the
face, hands and fingers are affected first. The skin
develops a diffuse, hard texture (sclero = hard; derma =
skin), with the surface becoming smooth. Later, internal
organs (e.g., the esophagus or lungs) become involved.
Diffuse scleroderma occurs throughout the entire body. It
often affects the skin and other parts of the body like
the lungs, heart, kidneys and blood vessels. Depending
upon the system involved, diffuse scleroderma may cause
such disorders as hypertension, muscle weakness,
swallowing difficulties, shortness of breath, and
difficulty grasping items or even typing. This diffuse
type of scleroderma progresses at different rates in
different people.
Many internal organs ultimately become involved with
diffuse scleroderma
TREATMENT. Aim treatment of scleroderma at
improving or at least maintaining ranges of motion of the
joints and decreasing pain as much as possible.
Physiotherapy under the guidance of an occupational or
physical therapist is most helpful and effective. This
includes movement exercises, medical massage therapy and
underwater massages, swimming, walking, cycling, paraffin
baths, chiropractic treatments, and the various types of
myofascial release techniques. All these activities help
keep the entire body flexible.
Perform specific exercises to keep the joints and skin
flexible. Practice the following exercises at least twice
daily:
Perform mouth-stretching exercise. Slowly stretch your
fingers on a table top, using firm and constant pressure.
Apply ice to the exercised areas when finished. Consult a
physical therapist, occupational therapist, or a
chiropractic physician who is trained in soft tissue
therapies for specific exercises
In addition to exercises, practice "joint
protection" procedures. Protect painful and swollen
joints--including the temporomandibular--from stresses
and injuries with some of the following suggestions:
Avoid lifting heavy objects (I limit my patients to
lifting no more than 10 pounds). During times of painful
and/or swollen TMJs, stick to soft foods and refrain from
chewing gum. Faithfully take the medicines recommended by
your doctor whether they are prescription or
over-the-counter drugs. Limit your activities; be
sensible when performing physical tasks. Consult the
United Scleroderma Foundation for further information, or
for a personal account with lots of encouragement,
contact Surviving Scleroderma
Systemic treatment includes the use of anti-inflammatory
medications such as non-steroidal anti-inflammatory drugs
(aspirin, ibuprofen, Aleve, Naprosyn), glucocorticoids,
and anti-fibrotic medications (penicillin infusions,
gamma-interferon, and D-penicillamine). Antacids may be
used to treat heartburn and to protect the lining of the
esophagus. Also, hypertensive medications for high blood
pressure and drugs which improve blood flow due to
Raynaud's symptoms may be prescribed.
Adhere to strict dietary changes. Eliminate caffeine and
refined sugars. Also, if oral manifestations occur,
maintain good oral hygiene and reduce foods with high
carbohydrate concentrations. See your dentist on a
regular basis
to avoid developing periodontal (gum) and tooth problems,
which will develop rapidly.
Protect your skin to maintain as good as blood supply as
possible to your extremities. Dress warmly in cold
weather. Wear a hat and cover your face and ears with a
scarf. Also, during winter months, use a humidifier to
keep moisture in the air of your home. Avoid strong
detergents and soaps which may irritate your skin and use
creams which moisturize your skin.
Attempt to manage your stress as stress makes the
symptoms of all chronic diseases, including scleroderma,
far worse. Be sure to get enough sleep, which may mean
taking short naps during the day. Consult a psychologist,
social worker or counselor for assistance in stress
management.
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This page was constructed with the assistance of Dr. Wesley Shankland, DDS, MS, PHD, TMJ & Facial Pain Center, Colombus, Ohio. We appreciate his generous donation of information for our web site at Suwanee Dental Care.