SCLERODERMA

(Systemic sclerosis)

Dr Sachidananda Mallya P P
SCLERODERMA

• chronic disease of unknown etiology

• affects the microvasculature and loose connective tissue

• characterized by fibrosis of the skin and internal organs.

 ETIOLOGY

• Autoimmune disease of unknown etiology

• Genetic – HLA DR3, DR 5, B8

• environment --- infectious agents like cytomegalovirus or parvovirus
B19, polyvinyl chloride, silica dust, epoxy resins

• The primary defect in scleroderma is still unknown, but three

pathologies have been identified:
 endothelial damage,
immunologic and inflammatory activation
Dysregulated extracellular matrix production.
CLINICAL FEATURES

• 19 persons per million population each year.

• Women -- three times more

• Adults – 30-50 yrs
• onset insidious

• cutaneous changes often responsible for bringing the problem

to the patient's attention.
• Often one of the first signs Raynaud's phenomenon ---a
vasoconstrictive event triggered by emotional distress or
exposure to cold.

• SS begins --- hands, face / trunk

• Skin :

Early typical indurated edema of skin

Usually accompanied by erythema

Progresses --- yellow/grey/ivory-white waxy appearance

diffuse, hard texture
Involvement of the facial skin by subcutaneous collagen deposiion
==== smooth, taut, cannot be wrinkled == masklike facies
• the nasal alae become atrophied, resulting in a pinched
appearance to the nose, ===="mouse" facies
• Resorption of the terminal phalanges and flexion contractures produce
shortened == clawlike fingers .

• The vascular events and the abnormal collagen deposition contribute to the
production of ulcerations on the fingertips
• Involvement of other organs may be subtle at first, but the results are
more serious.

• Fibrosis of the
 lungs
 heart
 kidneys
 GIT
=========leads to organ failure.

• Pulmonary fibrosis ---pulmonary hypertension and heart failure ---a

primary cause of death
Circumscribed / localized scleroderma (morphea)

• Excessive collagen deposition leading to thickening of the

dermis and subcutaneous tissues.

• Features such as sclerodactyly, Raynaud’s phenomenon, and

internal organ involvement are absent

• It can be further classified into plaque, generalized, linear,

bullous and deep subtypes
• Plaque Morphea
One or several, well circumscribed, oval or round, firm, indurated
plaques of variable size
Whitish/yellowish – surrounded by violaceous halo
 commonly on the trunk

• Bullous Morphea
Tense subepidermal bullae can occur overlying typical morphea
lesions.
• Linear Morphea
Single unilateral linear band, with hardening of the skin and
pigmentary changes.
The lilac color is obvious around advancing borders.
There may be deep atrophy and involvement of the muscle and
bone.
en coup de sabre --- fronto-parietal area
• Deep Morphea
inflammation and sclerosis in the deep dermis, fascia, or superficial muscle

• Generalized morphea
a combination of several of the types
 ORAL MANIFESTATIONS

• Microstomia --- collagen deposition in perioral tissues.

• limitation of mouth opening

• Characteristic furrows radiating from the mouth

produce a “purse string" appearance.

• Loss of attached gingival mucosa and multiple areas of gingival recession.

• Dysphagia --- deposition of collagen in the lingual and esophageal submucosa === firm ,
hypomobile (boardlike ) tongue and an inelastic esophagus,
• Xerostomia
 R/F
• diffuse widening of the periodontal ligament space is often present throughout the
dentition .

• Varying degrees of resorption of the posterior ramus of the mandible, the coronoid
process, the chin and the condyle resorbed

• because of the increased pressure associated with the abnormal collagen production
• Individual tooth resorption.
 HISTOPATHOLOGY

• Thickening and hyalinization of collagen fibres

• Atrophy of epithelium with loss of rete pegs

• Loss of dermal appendages – esp sweat glands

• Increased melanin pigmentation
• Sub- cutaneous fat disappears
• Walls of BV become sclerotic
 TREATMENT & PROGNOSIS
• Systemic medications, such as D- penicillamine, ---attempt to inhibit collagen
production.

• Controlling symptoms --- esophageal dilation to temporarily correct the

esophageal dysfunction and dysphagia .

• Calcium channel blocker ---increase peripheral blood flow and lessen the
symptoms of Raynaud 's phenomenon
• ACE inhibitors often effectively control hypertension if kidney involvement is
prominent.

• The prognosis is poor

• heart and pulmonary involvement - particularly poor.
• Approximately 80% of patients will survive 2 years after diagnosis