Pearls from Dr. Elana Bernstein

Young woman presenting with a digital ulcer found to have new systemic sclerosis

2016-06-22

  • Scleroderma = systemic sclerosis vs. localized scleroderma (morphea)

    Systemic sclerosis
    Limited cutaneous systemic sclerosis (lcSSc): skin involvement restricted to face and distal extremities
    Diffuse cutaneous systemic sclerosis (dcSSc): skin involvement proximal to distal forearms and knees

    Localized scleroderma (morphea)
    Typically circumscribed, generalized, linear skin lesions without internal organ involvement

    Posted 06/22/16 10:10:23 AM by Matthew Cummings

  • Palpable tendon friction rubs: pathogenesis unclear, likely due to fibrin deposition in tendon’s synovial sheath

    Posted 06/22/16 10:17:16 AM by Matthew Cummings

  • Nailbed capillaroscopy: presence of megacapillaries and decreased capillary density present in 60-80% of patients with lcSSc and dcSSc

    Posted 06/22/16 10:42:29 AM by Matthew Cummings

  • Extractable nuclear antigen (ENA) panel

    Anti-centromere: limited cutaneous systemic sclerosis

    Anti-RNA polymerase 3:  associated with rapidly progressive skin disease in diffuse cutaneous systemic sclerosis, increased risk for renal crisis, GAVE, and malignancy

    Posted 06/22/16 10:53:49 AM by Matthew Cummings

  • Scleroderma renal crisis
    Risk factors: +anti-RNA polymerase, diffuse cutaneous or rapidly progressive SSc, recent treatment w/ high dose corticosteroids

    Physiology: narrowing of arterial vessels --> renal hypoperfusion --> hyperplasia of JGM apparatus --> increased renin release

    Treatment: ACEI


    Posted 06/22/16 11:07:57 AM by Matthew Cummings

  • Raynoud's phenomenon treatment: dihydropyridine CCBs (nifedipine XL, amlodipine), ASA


    Posted 06/22/16 11:16:56 AM by Matthew Cummings

  • For further reading...

    Tendon friction rubs in SSc
    Nailbed capillaroscopy in SSc
    Scleroderma renal crisis

    Posted 06/22/16 11:18:55 AM by Matthew Cummings


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