Pearls from Dr. Elana Bernstein

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


  • 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

  • Created by Christopher Kelly
    Book be65e564ffbc09cd3ee32372501a5ece813f14b576dc711ff66870a4906e7f62
    Know a hypochondriac? Get them the best-selling book 'AM I DYING?!: A Complete Guide to Your Symptoms, and What to Do Next'
    The information on the website does not constitute official guidelines except where explicitly stated.
    It is not meant to replace the advice of a health professional.