This document discusses systemic sclerosis, also known as scleroderma. It begins with an introduction defining scleroderma as a chronic autoimmune disease affecting connective tissues, characterized by thickened skin. The etiology is unknown. It then describes the main clinical features of scleroderma, which can be remembered using the mnemonic "CREST" - calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. Scleroderma can affect multiple organs and cause difficulties with eating, swallowing, and reduced mouth opening. The document outlines the different types of scleroderma, epidemiology, pathology,

1. Systemic sclerosis
Kanwal fatima
Dental House officer
Isra dental college

2. Introduction
• It’s a chronic multisystem progressive disease
of Connective tissues. The thickened skin
distinguishes scleroderma from other
connective tissues.
• Etiology is unknown; insidious onset often
associated with Reynaud's phenomenon
(painful reversible digital ischemia on
exposure to cold).

3. Features
• Systemic sclerosis is sometimes referred to
as CREST. This is a mnemonic you can use to
remember some of the main symptoms of the
disorder:
• - C – Calcinosis – calcium deposits, usually
seen in the fingers
• - R – Raynaud’s phenomenon
• - E – Esophogeal Dysmotility
• - S – Scleodactyly – thickening of the skin
• - T – Telangiectasia – red spots on the skin

5. Clinical features
• Skin pigmentation changes – commonly a loss of
pigment around the affected areas, but there
may also be patches of hyperpigmentation.
• Calcinosis of the fingertips – calcium deposits at
the fingertips. You may see these at little pits
near the finger tips (digital pitting), and they
are often visible as white dots around the distal
phalanges on radiograph.

6. • Reynaud’s phenomenon – the result of vascular spasms that
reduce the blood supply to the fingers, usually when the hands
get cold. The phenomenon may also be triggered by emotional
stress. There is a classic pattern of colour change – the fingers
will go white, then blue, then as they warm up, or the episode
passes, they will become red. The red part of the cycle is the
result of hyperaemia that occurs after a period of reduced
blood flow. The episodes are often painful.
• o Sometimes also occurs in the tongue, toes, nose and ears.
• o Occurs in 4-30% of all women. Rarer in men

7. • Patients are at high risk of early pulmonary
fibrosis and acute renal involvement.
• Eating difficulty due to immobility of
underlying tissues.
• Dysphagia when the esophagus is involved.

8. Cntd.
• waxy mask-like face (mona-lisa face).
• Thickening of skin
• Involvement of multiple organs GI liver
kidneys TMJ;hence reduced mouth opening

9. Types
• 1- Limited cutaneous scleroderma –
aka scleroderma – in this variation, the signs are
mostly confined to the hands, arms and face – i.e.
mostly to the skin. In 80% there is also pulmonary
hypertension.
• o 5 year survival is >90%
• o 10 year survival is >75%
• o Generally only those with pulmonary
involvement with have life threatening illness
• o Usually skin changes on the upper limb
are distal to the elbow.

10. 2- Diffuse cutaneous scleroderma – aka systemic
sclerosis – tends to be more rapidly progressing
and severe. Affects larger areas of the skin, and
there is multi-systemic involvement. Can be life-
threatening, e.g. if the heart/lungs/liver/kidneys
become involved.
• o 5 year survival is 70%
• o 10 year survival is 55%
• o Skin changes can occur anywhere, and in
advanced cases, may cover the whole body!
• o Patches typically appear on the trunk

11. Epidemiology
• - 4x as common in women
• - Prevalence is about 1 per 1000
• - Peak incidence is between 30-50
• - Children sometimes affected in localised
patches

12. Pathology
• - The disease is the result of vascular
damage within the skin and organs.
• - Organ damage is usually the result
of fibrosis.
• - Renal and pulmonary complications are the
most life-threatening
• - In normal disease progression, there can
be some element of disease regression.This might
include periods, perhaps a few weeks long, where
the patient says their symptoms feel much less
severe – although they are usually still apparent.

13. Diagnosis
• Antibodies are present
• Circulating levels of E-selectin and
thrombomodulin are useful markers.

14. Treatment
• Combination of cyclophosamide and steroids
in early disease.
• In later stages  pencillamine but it causes
unwanted effects.

15. CASE REPORT
• A 58 years old female edentulous patient
presented to the department of
Prosthodontics for a set of dentures. She was
a diagnosed case of systemic sclerosis and a
known case of hypertension for more than
four years on regular treatment. Her general
physical examination revealed hardening of
facial skin, vertical peri-oral furrows, thinning
of lips with reduced mobility

16. • Showing extra-oral features

17. • Showing resorption of distal phalanges

18. Procedure
• The smallest diameter stock tray was chosen
for preliminary impression, but still it was not
possible to insert the tray loaded with
impression material inside the patient’s oral
cavity. Hence, the modified impression
technique was adopted. The metal stock tray
was sectioned antero- posteriorly following
the line passing to the left side of the midline.

19. • Showing sectional mandibular stock tray.

20. • The impression of the right half was made and
the left section of the loaded impression tray
was inserted before removing the other half.
The two parts of the impression were
removed in the reverse order. Impression was
assembled outside the mouth to get the
primary cast. A similar technique was followed
for the secondary impression with lock and
key mechanism created in the handle of the
sectioned custom tray.

21. • Showing sectional maxillary custom tray

22. • The assembled final impression was poured to
get the master cast. Conventional method for
preparation of occlusal rims was followed.

23. • Reduced mouth opening limited the use of Fox plane
guide for the registration of occlusal plane. So, two
metal scales were used on each side to access the
occlusal plane. The visual methods are more significant
in such patients than conventional and the anatomical
landmarks were used as a guide. Try-in was done and
denture was processed using compression molding
technique. Patient was trained to use a rotational path
of insertion and removal. She was advised to
frequently sip water or non-sugary fluids for
xerostomia. The cosmetic and functional result of the
final denture was good.

24. • Showing final prosthesis.

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