Dena J. Fischer, DDS, Lauren L.
Patton, DDS
Scleroderma: oral manifestations and treatment challenges
Scleroderma is a connective tissue
disorder of excess collagen production
characterized by intense fibrosis of the
skin, with internal organ involvement.
A wide range of oral sequelaeincluding
microstomia, oral mucosavgingival
fibrosis, xerostomla, and mandibular
bone resorptioncomplicates mainte-
nance of oral health and dental treat-
ment. The literature is reviewed, and
two cases are presented.
240 SCD Special Care In Dentistry, Voi20 No 6 2000
cleroderma is a chronic, multi-
S system disorder of connective
tissue characterized by fibrosis
of the skin, blood vessels, and other
organ systems, including pulmonary,
renal, gastrointestinal, and cardiac.
This disease presents with a wide
clinical picture that varies greatly in
severity and prognosis. This connec-
tive tissue disease has been divided
into two main subsets, systemic scle-
rosis and localized scleroderma.
Localized scleroderma is usually
limited to skin lesions, sometimes
involving the underlying muscle and
bone. It has been further classified
into morphea with five subgroups:’
plaque-like, generalized, bullous, lin-
ear (including en coup de sabre), and
deep. The prevalence of localized
scleroderma is 2200/million, with an
incidence rate of 27 new cases/mil-
lion population per year.2
In 1980, the American College of
Rheumatology3 created a classification
scheme for systemic sclerosis, charac-
terizing the disease as diffuse or limit-
ed. Systemic sclerosis with diffuse cuta-
neous scleroderma, previously known
as progressive systemic sclerosis, is
identified by the rapid development of
symmetric skin thickening of proximal
and distal extremities, face, and trunk,
with visceral organ involvement.
Systemic sclerosis with lLmited cuta-
neous scleroderma presents as skin
thickness confined to distal extremities
and the face. The limited form fre-
quently has the features of the CREST
(calcinosis,Raynaud’sphenomenon,
esophageal involvement, sclerodactyly,
telangiectasias)syndrome and involves
internal organs less often than diffuse
cutaneous scleroderma.
The prevalence of systemic sclero-
sis is approximately 250/million pop-
ulation in the United States, far higher
than in other countries,2,4with an inci-
dence rate of approximately 19 new
cases/million per year.5.6 There is a
female preponderance, ranging from
3:l to 14:l in the childbearing years
with peak incidence occurring in the
third through fifth decades.2~5.7It is
seen more frequently and presents
more severely in African-Americans
than in other ethnic groups.4,7,8While
the etiology of this disease is
unknown, evidence suggests a weak
genetic component that interacts with
an environmental trigger (or triggers)
for disease expression.2
Disease pathophysiology
The pathogenesis of scleroderma
involves the processes of fibrosis,
inflammation, and vascular dysfunc-
tion. A mediated inflammatory cell
reaction in scleroderma patients leads
to an infiltration of T-cells and elevated
levels of circulating cytokines, such as
interleukin. Some of these circulating
products activate fibroblast activity and
bind to endothelial cells. These activat-
ed fibroblasts release cytokines and
growth factors and ultimately overpro-
duce collagen and other extracellular
matrix products while exhibiting
decreased collagenaseproperties.gJ0
Inflammatory activity may also con-
tribute to the altered endothelial per-
meability and abnormal function of
blood vessels, leading to hypoxia,
ischemia, and intravascular occlusion.”
While antinuclear antibodies are pre-
sent in more than 85%of scleroderma
patients,lz they are not directly related
to the pathogenesis of the disease.
Clinical manifestations
The onset of systemic sclerosis usually
begins with Raynaud's phenomenon,
characterized by blanching and
cyanosis of digits resulting from
vasospasm in structurally diseased
blood vessels in response to cold or
emotional stress.13Raynaud's may last
for many years, such as in limited scle-
roderma, or may present with a short
duration before more symptoms
develop, as in diffuse scleroderma.
The initial edematous phase is charac-
terized by arthralgias and swelling of
the whole hand as well as the feet.
During the indurative phase, skin
becomes firm, thickened, and tightly
bound to the underlying subcuta-
neous tissue. This progresses up the
extremities and the trunk before
involving internal organs. After 3 to 4
years of fibrosis, disease progression
appears to stabilize, and patients often
experience an improvement in their
skin thickening.14 However, organ
system damage that is already present
can continue to cause debilitating
problems.
In diffuse scleroderma, rapid devel-
opment of hand swelling and skin
tightening (sclerodactyly)often leads
to severe flexion contractures with
clawlike hand deformities and serious
di~abi1ity.I~ Patients may have a firm
mask-like facial appearance, with a
pinched nose resulting from nasal alar
atrophy. Severe muscular, gastro-
intestinal, pulmonary, renal, and car-
diac conditions also result from inter-
nal organ fibrosis and muscular atro-
phy. Scleroderma-relatedpulmonary
disease is the most frequent cause of
death in these patients.15
Pharmacologic treatment of the
disease has been attempted with D-
penicillamine and colchicine, which
are thought to help prevent progres-
sion of the fibrotic process. Clinically,
however, these disease-moddying
drugs have exhibited only minimal
success.16-18More effective therapies
have been identified to manage organ-
based manifestations of the disease.19
Oral findings
Systemic sclerosispresents with a wide
range of oral mamfestations. A reduced
oral opening occurs in 7040% of these
patients?@" resulting from collagen
deposition in the skin and peri-oral tis-
sues. Telangiectasias of the oral mucosa
are evident in 56%of patients."
Furthermore, collagenous changes of
the oral mucous membranes cause
them to appear thin, pale, and tight,
with ulcerations resulting from loss of
vascular integrity.23.24 This mucosal
fibrosis may also induce gingival reces-
sion and stripping of the attached gin-
giva.20 This fibrotic process induces cre-
nations of the tongue and buccal
mucosa in 25% of patients.20 Moreover,
intense fibrosis of the tongue, soft
palate, and larynx may cause limited
mobility and consequently dysphagia
for affected individuals.25
About one-third of patients with
scleroderma exhibit decreased
lacrimal and salivary secretion
rates,20,22t26which could be due to a
purely fibrotic process or to sec-
ondary Sjogren's syndrome.22
Biopsies of minor salivary glands
have shown fibrosis of the glandular
tissue with endothelial cell and capil-
lary basal membrane damage.22
Radiographically, an increase in
the thickness of the lamina dura has
been e~ident~l,~3,~7,2* and is often
more pronounced in the posterior
teeth.27 Microscopic examination of
thickened periodontal ligament
spaces found thickening of the colla-
gen walls with accompanying nar-
rowing of the blood vessel lumina in
the periodontal membrane.29.30 Teeth
exhibiting this radiographic finding
are not mobile and have intact gingi-
val attachments.31
Bone resorption has been
observed at the angle of the
mandible, as well as the coronoid
process and the condyle,21,24,27,32
probably caused by pressure atrophy
secondary to ischemia. In some cases,
the resorption has been so severe as
to cause pathologic fracture of the
mandible.27These condylar changes
may result in the temporomandibular
joint symptoms of popping, clicking,
and crepitus.20
Case history #l
A 20-year-old Caucasian male, who
was diagnosed with dermatomyositis
and scleroderma (overlap syndrome)
at age 6, presented to the University
SCD Special Care In Dentistry, Voi20 No 6 2000 241
of North Carolina (UNC) hospital
dental clinic. Dermatomyositis is an
idiopathic inflammatory disorder of
striated muscle and the overlying
skin associated with symmetric proxi-
mal muscle weakness and atrophy.
When the disease was active, from
ages 7-14, his muscular growth was
delayed, and he was treated with
high-dose steroid therapy. The
patient stated that his dermatomyosi-
tis has been in remission, and that he
had actually grown 2-3 inches in the
previous year. He presented with a
small stature, hyperkeratosis of his
joints, particularly the knees, and
sclerodactyly with atrophy of his fin-
gertips (Fig. 1). His oral opening
allowed lip-to-lip opening of about 30
mm, with a 21-mm interincisal dis-
tance (Fig. 2).
Intra-orally, this patient had poor
oral hygiene, with extensive decay,
moderate alveolar bone loss, and gin-
gival recession on the facial aspects of
his mandibular anterior teeth, where
the skin tightness was pulling on the
intra-oral mucosa (Fig. 3). He had a
Class I11 occlusal relationship with an
anterior crossbite.
Limited intra-oral access was a
significant impediment to dental
treatment. The patient was initially
taught facial grimacing and oral aug-
mentation exercises, including pro-
gressive stacking of tongue depres-
sors between his incisors, to cope
with his microstomia. Such proce-
dures enabled him to increase his
interincisal distance 3 mm, which
was sufficient to allow for treatment
with a pediatric handpiece and
extraction forceps. Multiple facial and
interproximal composite resin and
amalgam restorations were complet-
ed, and grossly decayed teeth were
extracted without complications (Fig.
4). Nitrous-oxide/oxygen sedation
was required to overcome his anxiety
during the first three treatment ses-
sions, until his coping skills
improved. Once treatment was com-
pleted, the patient was placed on a
six-month hygiene recall schedule,
and fluoride trays were fabricated to
allow for daily application of 1.15%
neutral sodium fluoride. Limited
manual dexterity, resulting from the
sclerodactyly, continued to hamper

Flg 1.Case # l:
Sclerodactyly. Involvement of the skin overlying the digits compromises
oral hygiene.
his daily oral hygiene efforts. No
prosthodontic treatment was planned
for edentulous areas. After two years,
the patient was lost to follow-up.
Case history #2
A 49-year-old Asian female presented
to the UNC hospital dental clinic with
a history of systemic sclerosis with
diffuse cutaneous scleroderma diag-
nosed four years previously. The dis-
ease progression caused skin fibrosis,
pulmonary complications resulting in
a reduction in total lung capacity and
vital capacity, peptic ulcer disease
Flg2. Case #l Mlcrostomia, wlth reduced
lnterlnclsal distance, and atrophy of the nasal
alae, creatinga plnched nose appearance.
242 SCD Special Care In Dentistry,Vol20 No 6 2000
with severe gastrointestinal reflux,
and multiple kidney stones.
Furthermore, an electrocardiograph
disclosed left atrial enlargement.
She had severe microstomia (25-
mm opening), a severely atrophic
mandible, limited TMJ mobility, and
bilateral resorption of the mandibular
angles and condyles (Fig. 5). She also
had a thinning nose and sclerodacty-
ly with atrophy and ulceration of her
digits. An evaluation of a sagittal
coronal radiograph revealed atrophy
of the parotid, submandibular, and
lacrimal glands, with calcifications
Flg 3. Case # I recession with no attached ghglval faclal-to-central incisors.
Ginglval
noted in both parotid glands.
Intra-orally, the patient had poor
oral hygiene, gross decay, and gen-
eralized severe periodontal disease
with tooth mobility. In addition to
xerostomia, a thin, tight mucosa
was noted bilaterally in the posteri-
or buccal vestibules. Given the non-
restorable condition of her denti-
tion, she underwent a full-mouth
extraction under general anesthesia,
she was warned of a significant risk
of mandibular fracture due to
severe atrophy of the ramus and
angles (Fig. 6). Because of poor
alveolar bone support, no teeth
could be saved for partial or over-
denture abutments.
Maxillary and mandibular com-
plete dentures were fabricated with
minimal interocclusal space to allow
for good esthetics and sufficient
function. Preliminary alginate
impressions were made with modi-
fied standard trays. Custom trays
and rubberbase impression materials
were used for the final impressions.
The maxillary final impression was
repeated in an attempt to improve
retention. There was minimum reten-
tion on the mandibular complete
denture due to severe mandibular
atrophy. The patient has been able to
insert and remove the dentures suc-
cessfully on her own. To date (6
months' follow-up), the patient is
pleased with the esthetics, function,
and comfort of the dentures and has

Flg4. Case #l (a) Initial panoramic radiographlc presentation (January, 1991). (b) Post-
treatment panoramic radiograph (April, 1994).
tried to comply with the precaution
against forceful mastication.
Discussion
Scleroderma patients present with a
wide range of oral findings. Due to
the unique manifestations of this dis-
ease, dental management of these
patients requires a multidisciplinary
approach. Early intervention is
important, given the progressive scle-
rosis that occurs in many patients.
Mouth-stretching and oral aug-
mentation exercises have been proven
successful in increasing mouth open-
ing by 3-5 mm in microstomic
patients.28 Bilateral commissurotomy,
a surgical procedure used to increase
the size of the labial opening, may be
indicated in selected patients.
However, given diminished repara-
tive processes associated with this
condition, surgery carries the risk of
poor wound healing and facial scar-
ring. This reduced oral opening is of
clinical significance,because it impairs
the individual's ability to maintain
oral hygiene and makes dental proce-
dures and treatment difficult.
Exercises to increase mouth opening
have been proven effective in our
male patient to allow access for
restorative dental procedures without
the need for commissurotomy.
Xerostomia is a common finding
with scleroderma patients, occurring
as a result of fibrosis of the salivary
glands.22.24 Dry-mouth symptoms can
cause a high caries rate and an
increased incidence of candida infec-
tions. Dysphagia may also result
from a decreased salivary flow as
well as from tongue fibrosis and lim-
ited tongue mobility.33
For the management of xerosto-
mia, sugarless candies and mints and
pharmacological agents (it?.,pilo-
carpine) may be used to stimulate
salivary flow, and salivary substitutes
may improve comfort. Also, frequent
hygiene visits, as well as a customized
oral hygiene program such as adap-
SCD Special Care In Dentistry, Vol20 No 6 2000 243
Flg8. Case #2: Patlent profile showlng
resorption of the angle of the mandible.
tive equipment, electric toothbrushes,
and oral opening maintenance exer-
cises, are recommended, since these
patients often lack the manual dexter-
ity to maintain good oral hygiene.
Furthermore, daily fluoride treat-
ments with the use of custom fluoride
trays are useful for caries control.
Decreased salivary flow can also
be problematic for patients who
require removable prostheses, which
can cause a reduced tolerance to pres-
sure generated by the prostheses.
Tongue rigidity inhibits mandibular
denture control.34 Moreover, atrophic
mandibles prevent adequate reten-
tion of mandibular prostheses, and
heavy occlusal forces on the mandible
may result in a probability of patho-
logic fracture. For patients with ade-
quate bone levels, implant-supported
fixed35f36and removable prosthe-
ses34~37have been reported in the lit-
erature. However, many patients lack
the mandibular bone height required
for consideration as implant candi-
dates and are also not good surgical
candidates for bone-grafting proce-
dures. Denture wearers need fre-
quent recalls to allow for denture
adjustments and evaluation of the
mucosa's ability to withstand forces
without further atrophy. Denture fab-
rication and use were complicated in

fig 6. Case #2: Panoramic radiograph demonstratlng bilateral mandibular angle and
ramus resorption.
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