Systemic sclerosis on finger

Diagnosis: Systemic sclerosis on finger

Systemic sclerosis on finger

Clinical Presentation

Systemic sclerosis on finger

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

Tags: systemic, sclerosis, finger, ellie goulding md