Dentomaxillofacial Radiology (2005) 34, 384386

q 2005 The British Institute of Radiology

http://dmfr.birjournals.org

CASE REPORT

Mandibular resorption in progressive systemic sclerosis:

a report of three cases
A Auluck*,1, KM Pai1, C Shetty2 and SD Shenoi3
1
Oral Medicine and Radiology, Manipal College of Dental Sciences, Manipal, India; 2Department of Radiodiagnosis and Imaging,
Kasturba Medical College, Manipal, India; 3Department of Dermatology, Kasturba Medical College, Manipal, India

Progressive systemic sclerosis is a generalized collagen disorder, which is characterized by fibrosis

that involves skin, muscles and other organ systems like the gastrointestinal tract, lungs, heart and
kidneys. Its oral manifestations include features like restricted mouth opening, widening of
periodontal ligament space, pseudoankylosis, malocclusion and mandibular resorption. Mandibular
resorption in systemic sclerosis is relatively uncommon and is reported only in 10% of cases. The
purpose of reporting these three cases is to highlight the importance of screening all patients with
advanced systemic sclerosis with panoramic radiographs. Panoramic radiographs are essential for
early detection of resorption in the mandible to prevent possible consequences like pathological
fractures, osteomyelitis and neuropathies.
Dentomaxillofacial Radiology (2005) 34, 384386. doi: 10.1259/dmfr/14556986
Keywords: mandible, bone resorption, systemic sclerosis, panoramic
Case reports
Case 1
A 24-year-old woman with an 8 year history of systemic
sclerosis presented with a complaint of difficulty in
opening the mouth for the past 2 years. She was unable
to open the mouth wide and experienced pain and clicking
sound in the left temporomandibular joint region. Pain was
moderate, radiating to the neck and aggravated on opening
the mouth wide. On opening the mouth, her jaw deviated to
the left side.
On intraoral examination, the patient was partially
edentulous. Periodontal status was good and no abnormal
mobility of teeth was observed.
Physical examination showed a thin woman with
classical orofacial signs and symptoms of systemic
sclerosis like tightening of the skin around her mouth,
thin atrophic lips, microstomia and limited mouth opening.
The patient had tight firm skin with claw like deformities
of the hands. A panoramic radiograph was advised which
revealed resorption of the right coronoid process and
shortening of the left condylar neck (Figure 1).

*Correspondence to: Dr Ajit Auluck, Department of Oral Medicine and Radiology,

Manipal College of Dental Sciences, Manipal 576104, Karnataka, India;
E-mail: drajitauluck@yahoo.co.in
Received 18 August 2004; revised 11 April 2005; accepted 1 May 2005

Case 2
A 34-year-old female patient, a confirmed case of systemic
sclerosis for the past 5 years, was referred to our
department for routine oral examination and for consideration of denture construction. She did not report any oral
problems. She had undergone complete extraction of teeth,
as they were mobile.
On examination, there was loss of pliability of skin,
restricted mouth opening and thin atrophic lips. The face
appeared narrow. A screening panoramic radiograph was
advised which revealed resorption of bilateral mandibular
angle and posterior border of the mandible (Figure 2).
Case 3
A 12-year-old girl with a confirmed case of systemic
sclerosis was referred for evaluation of facial asymmetry
and painless progressive fibrosis of the cheek for the last
year. There was no history of trauma or any infectious
disease.
On examination, the face was asymmetric with
hypoplasia of the left side of the lower face. An indurated
area of 6 cm 2 cm was observed over the left cheek. It
extended from the corner of the mouth to the preauricular
region and vertically from ala tragal line, crossing over the
body and inferior border of the mandible, extending to the
left submandibular region. Induration was also observed
over the skin of hands and feet. There was loss of pliability

Mandibular resorption, systemic sclerosis

A Auluck et al

385

canal was apparently positioned at the lower margins of the

resorbed mandible.
Discussion

Figure 1 Panoramic radiograph of case 1, resorption of the right

coronoid process and shortening of the left condyle with degenerative
changes on left condylar head are shown

Figure 2 Panoramic radiograph of case 2, showing resorption of

bilateral mandibular angle and posterior border of ramus of the mandible

of overlying skin with atrophy of muscles and subcutaneous tissues. Facial motor and sensory functions were
normal. Intraoral examination revealed over-retained left
upper and lower deciduous canines. Teeth and periodontium were healthy. Mouth opening was reduced.
A panoramic radiograph was advised which revealed
resorption of the left lower border and angle of the
mandible with decrease in the length of the left ramus
(Figure 3). Development of the mandibular second molar
on left side was delayed in comparison with development
of the right second molar teeth. Due to the resorption of the
lower border of the mandible, the inferior alveolar nerve

Figure 3 Panoramic radiograph of case 3, showing resorption of the left

lower border of the mandible with decrease in the length of the left ramus

Progressive systemic sclerosis is a chronic generalized

collagen disorder of unknown aetiology. It is more
common in women than men and is characterized by
induration of the skin.1 The disease can occur in both
systemic or localized forms.2,3 Generalized or progressive
systemic sclerosis is characterized by tautness of the skin
and muscles, compromised function of the lungs, heart,
kidneys, and gastrointestinal tract and osteolytic changes
in the skeleton. Acrosclerosis is another form of systemic
sclerosis with a combination of scleroderma of extremities
and Raynauds phenomenon. The localized form of the
disease can present as morphea or en coup de sabre, which
differs from systemic sclerosis by the absence of systemic
involvement.4 Morphea or circumscribed scleroderma is
characterized by local changes limited to the skin, which
assumes a thickened, hidebound character. All of these
patients were confirmed cases of progressive systemic
sclerosis having facial and multisystem involvement.
There are two syndromes associated with systemic
sclerosis: CREST syndrome that is characterized by the
presence of calcinosis cutis, Raynauds phenomenon,
oesophageal hypomobility, sclerodactyly and telangiectasia; and Thibierge Weissenbach syndrome, which presents with extensive subcutaneous tissue calcinosis. None
of our patients presented with features suggestive of either
of these syndromes.
Mandibular resorption in systemic sclerosis has a
multifactorial aetiology. Rout et al1 and Haer et al5
reported the incidence of mandibular resorption in
systemic sclerosis to be 10% and 20% to 33%, respectively. Resorption of the mandible, terminal phalanges of
the hand, and distal portion of the radius and ulna are some
radiographic findings in patients with systemic sclerosis.3
Specific radiographic findings involving the mandible
include widening of periodontal ligament space and
resorption involving the ascending ramus, condyle,
mandibular angle, coronoid process and antegonial notch.
The angles of the mandible are most frequently involved
followed by condyle, coronoid process, and the posterior
border of the ramus.5 In case 1 there was resorption of the
right coronoid process with shortening of the left condylar
neck, while in case 2 there was bilateral resorption of angle
and posterior border of the mandible, and in case 3 there
was resorption of the left lower border and angle with
decrease in the length of the left ramus of the mandible.
It appears unusual that resorption in systemic sclerosis
is confined to the areas of muscle attachment (masseter,
pterygoids and temporalis), which are normally the last
areas of mandible to undergo physiological resorption.6 In
systemic sclerosis, there is increased fibrosis of muscles so
the muscles become bulkier. Simultaneously, there is a
decrease in the vascularity of the muscles due to
constriction of the vessels secondary to the fibrosis of
muscular walls of the arteries. Therefore, muscles undergo
Dentomaxillofacial Radiology

Mandibular resorption, systemic sclerosis

A Auluck et al

386

atrophy and exert pressure on the bone at the site of their

attachment causing bone resorption. In case 1, resorption
of the coronoid process can be attributed to the atrophic
changes in the temporalis muscle, which is attached to the
coronoid process.
Ischaemia of the bone can also be a factor secondary to
vasculitis associated with progressive systemic sclerosis
causing mandibular resorption.2,6 The connective tissue
abnormality associated with the disease can cause
impingement of the blood vessels.6 Ramon et al7 suggested
that the areas commonly showing resorption (condyle,
coronoid and angle) are supplied by small arterial branches
of the internal maxillary artery rather than the main inferior
alveolar artery. Progressive systemic sclerosis may preferentially affect these smaller arterial branches, compromising the vascularity and causing bone resorption.
Therefore, compromised vascularity together with the
atrophy of the lateral pterygoid muscle can explain the
shortening the left condylar neck in case 1.
Mandibular resorption can also be attributed to the
tightness of the skin causing pressure resorption of the
bone.6 Tight musculature exerts a continuous pressure on
the supporting mandible. This pressure further increases
during the functional jaw movements as the stiff muscles
limit mandibular movement. In case 2 and case 3, pressure

from the tight facial musculature resulted in smooth

asymptomatic resorption of the mandible.
In young patients the taut facial musculature restricts
mandibular growth, which can impair the development
of tooth buds. This explains the presence of over
retained deciduous teeth and delayed development of
left second permanent molar in case 3.
These areas of mandibular bone resorption are risk
zones for pathological fractures, osteomyelitis, apertognathia7 and trigeminal neuropathy3 causing severe pain to
the patients. In case 2, the patient was completely
asymptomatic but mandibular resorption was detected on
the screening panoramic radiograph. This emphasises the
need for radiographic examination in all patients with
systemic sclerosis. Follow up of such patients with
periodic panoramic radiographs is essential to monitor
and intercept potential consequences like pathological
fractures and neuropathies.

Acknowledgment
We thank Dr KS Bhat for his support and guidance in preparing
this article.

References
1. Rout PG, Hamburger J, Potts AJ. Orofacial radiological manifestations
of systemic sclerosis. Dentomaxillofac Radiol 1996; 25: 193 196.
2. Rubin MM, Sanfilippo RJ. Resorption of the mandibular angle in
progressive systemic sclerosis. Case report. J Oral Maxillofac Surg
1992; 50: 75 77.
3. Fischoff DK, Sirosis D. Painful trigeminal neuropathy caused by
severe mandibular resorption and nerve compression in a patient with
systemic sclerosis: case report and literature review. Oral Surg Oral
Med Oral Pathol Oral Radiol Endod 2000; 90: 456 459.
4. Demir Y, Karaaslan T, Aktepe F, Yucel A, Demir S. Linear
scleroderma en coup de sabre of the cheek. J Oral Maxillofac
Surg 2003; 61: 1091 1094.

Dentomaxillofacial Radiology

5. Haers PE, Sailer HF. Mandibular resorption due to systemic sclerosis:

case report of surgical correction of a secondary open bite deformity.
Int J Oral Maxillofac Surg 1995; 24: 261 267.
6. Pogrel MA. Unilateral osteolysis of the mandibular angle and coronoid
process in scleroderma. Int J Oral Maxillofac Surg 1988; 17: 155 156.
7. Ramon Y, Samra H, Oberman M. Mandibular condylosis and
apertognathia as presenting symptoms in progressive systemic
sclerosis (scleroderma). Pattern of mandibular bony lesions and
atrophy of masticatory muscles in PSS, presumably caused by affected
muscular arteries. Oral Surg Oral Med Oral Pathol 1987; 63:
269 274.