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Scleroderma

Scleroderma
Etiology • Autoimmune, with local and systemic or multiorgan effects
secondary to excessive collagen deposition • Localized sclerosis (morphea) is a distinct disorder sharing only
histologic features with systemic sclerosis.
Clinical Presentation • Oral findings in progressive systemic sclerosis are as follows:
• Limited oral opening (microstomia) • Narrowing of lips (“purse string” sign) • Raynaud’s phenomenon (an early finding) • Facial skin becomes taut and mask-like. • Tongue becomes “bound down” and hypomobile. • Telangiectases over facial skin, lips, tongue
Radiographic Findings • Prominent antegonial notch on panoramic radiograph • Variable resorption of condyles and coronoid processes • Uniform widening of periodontal membrane space • Root resorption
Diagnosis • Clinical features • Microscopic features of skin or mucosal biopsy • Serology: demonstration of anticentromere antibodies or anti-

topoisomerase I (anti-Scl 70)
Differential Diagnosis • Lichen sclerosus • Submucous fibrosis • Postradiation scarring
Treatment • Systemic corticosteroids • Immunosuppressive agents • Vasodilators
• Systemic d-penicillamine • Control of local effects of disease
Prognosis • Dismal for systemic form