SYSTEMIC SCLEROSIS

Diagnosis: SYSTEMIC SCLEROSIS

Case submitted by Dr.Azeem Alam Khan to the dermRounds community.

Clinical Presentation

Clinical photograph(s) submitted for peer review and discussion.

Clinical History

Submitted by Dr.Azeem Alam Khan. Originally posted September 29, 2009.

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, dr.azeem alam khan