Scleroderma

Diagnosis: Scleroderma

Scleroderma

Clinical Presentation

Scleroderma

Clinical History

Submitted by Ellie Goulding MD. Originally posted January 23, 2017.

Treatment

See case discussion.

Differential Diagnosis

• Morphea (localized scleroderma) • Eosinophilic fasciitis • Nephrogenic systemic fibrosis • Scleredema • Scleromyxedema • Mixed connective tissue disease • GVHD (chronic sclerodermatous)

Key Learnings

• Systemic sclerosis: skin fibrosis + internal organ involvement (lungs, kidneys, GI, heart) • Two subtypes: limited (CREST — formerly; anti-centromere) and diffuse (anti-Scl-70/topoisomerase I) • CREST: Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly, Telangiectasia • Raynaud phenomenon is often the earliest manifestation (years before other features) • Nailfold capillaroscopy: dilated, tortuous capillary loops with dropout — characteristic finding • Scleroderma renal crisis: hypertensive emergency — ACE inhibitors are life-saving • Anti-RNA polymerase III: associated with scleroderma renal crisis and cancer risk

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