Download - Management of the Skin with Scleroderma
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Management of the Skin in Scleroderma
Lauren Graham, MD, PhDClinical InstructorNorthwestern Department of DermatologyScleroderma Patient Education ConferenceOctober 10, 2015
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No disclosures•Off-label uses of medications will be discussed today•Always discuss with your personal physician
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Outline•Telangiectasias •Dry skin/itching•Raynaud’s•Digital ulcers•Calcinosis
•TIPS!!!
•Everyone is different, everyone’s body is different, everyone’s disease is different!
•Talk to your physician if any questions
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Functions of Skin
•Protection: ▫Keeps the insides in, the outside
out! ▫Temperature regulation
•Cosmetic: ▫Our first impression to the world
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Telangiectasias
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Telangiectasia•What are telangiectasias?
▫Small, dilated blood vessels near the skin▫Broken capillaries▫See them in localized and diffuse▫No longer functioning
•They can correlate with development of pulmonary hypertension, increased right ventricular systolic pressure
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Treatment of telangiectasia: Lasers/light
•What is a laser?▫Source of high energy light, used in many
dermatological conditions
•Two treatments that have been used to treat vascular lesions: ▫IPL: intense pulsed light▫PDL: pulsed dye laser
•Light/laser therapy: causes damage to vessel and forces it to collapse and scar down so blood can no longer flow through the vessels
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PDL (pulsed dye laser) and IPL (intense pulsed light)
•Both are effective, some studies show PDL is better•Treatment cannot prevent new ones from occurring•Skin of color: settings need to be less aggressive so
takes more treatments, risk of depigmentation•Side effects:
▫Pain (feels like a rubber band snap)▫Bruising (more aggressive treatment has increased risk)▫Redness, swelling, itching▫Rarely: infection, burn, changes in skin pigmentation
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PDL (pulsed dye laser) in scleroderma vs healthy patients
•16 patients with scleroderma and 20 healthy patients with sporadic telangiectasia were treated with PDL
•To achieve effective telangiectasia clearance: ▫Patients with scleroderma took an average of 3.24
treatments▫Control patients took average 1.92 treatments
•Possibly because of thicker skin and/or thickened blood vessels?
Halachmi, 2014
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TIPS for what to expect for treatment• Cost: ~ $500-1000 for one treatment, packages available
▫Pricing depends on how much area treated▫Insurance usually doesn’t cover but physician can try to fight it, but no
promises
• What to expect: ▫Everyone in the room will wear goggles, yours will be opaque▫Area will be cooled with the laser device, often given ice pack afterwards
• Time: depends on how many you want treated but pretty quick (< 5 min)
• May need 1-6 treatments, often 2-3; usually see improvement after 1
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•Don’t go to any laser treatment with a tan! And no tanning afterwards!
•Do NOT wear makeup to visit•Can apply Vaseline or moisturizer to help healing•Don’t pick if anything crusts•Can start wearing makeup after 24-48 hrs, ask your
doctor•Small study of 27 patients showed that patients on
bosentan had more new telangiectasias than on ilomedin
TIPS for what to expect for treatment
Hetzer, 2014
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Cosmetic cover ups• Cover FX: www.coverfx.com
▫ available at Sephora downtown
• Dermablend: www.dermablend.com▫ Camo confessions
• Cinema Secrets: www.cinemasecrets.com/-professional-cosmetics/
• Microskin: simulated second skin that is designed to your skin color▫ available in NYC or California ▫ Would need to go for consultation
• MAC
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Dry skin/itch
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Dry skin/itch•Dry skin itches!!•In fibrotic skin, you lose sweat glands and
sebaceous glands•Importance of emollients and dry skin care
•Avoid scratching---scratch/itch cycle•DO NOT scratch with backscratcher, sharp
objects▫Just makes it worse, opens skin and provides
avenue for infection•Minimize stress: lifestyle changes, yoga,
meditation, taking bath with music, avoid alcohol
Scratch
Itch
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• Itch: Use temperature to help you▫Ice-pack, frozen vegetables from the
freezer ▫Keep your moisturizer in the refrigerator ▫If you are not near a refrigerator or freezer,
you can also apply a cold water compress to calm the itch and then moisturize afterwards
• Shower once daily at the most, maximum 5-10 minutes long
• Warm or cool water, NO HOT WATER
Dry skin/itch
Scratch
Itch
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Dry skin TIPS!• Soaps: mild such as Dove Original Unscented or Cetaphil
cleanser
• Avoid soaps with fragrances, harsh soaps, scrubs, anything with beads or exfoliators in them
• Avoid anti-bacterial soaps, such as Dial or Lever 2000, unless instructed to use by the doctor
• Look for products that say “fragrance-free” NOT “unscented”, which allows for fragrances to still be used as ingredients as long as the final product doesn’t “smell”
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•Moisturize a lot:▫Apply moisturizer immediately after the shower
•Apply moisturizer to hands after each hand wash•Ointments are the most effective moisturizers. Creams
are less effective. Lotions are the least effective. ▫If in a pump form—too thin; need jar or tube
•Cerave, Vanicream, Aveeno, Eucerin (products have levels of thickness on the label)
Dry skin TIPS continued!
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• Original Vaseline (petroleum jelly) is cheap (1 pound jar for ~ $3-5) ▫Has the fewest ingredients which makes it the least
likely to cause any skin allergies▫Petroleum jelly has been shown to be as effective or
more effective than many of the expensive moisturizers on the market
▫Alternatives include Aquaphor ointment, Neutrogena Norwegian formula, Eucerin, Aveeno, Cerave
• Petroleum jelly can be sticky and can get on clothing; an ideal time to use petroleum jelly is at night and cover with old pajamas, so that it doesn’t get on clothing
Dry skin TIPS continued!
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For severe itch• Sarna Sensitive (pramoxine) is over the counter• Topical corticosteroids
▫Can mix with Eucerin or CeraVe cream• Tacrolimus ointment if skin is fragile
• Anti-histamines ▫Allegra, Claritin, Zyrtec, Benadryl, hydroxyzine (Atarax)▫May help but often does not help scleroderma itch
• Gapabentin (Neurontin), pregabalin (Lyrica) can be helpful▫Start low, increase as tolerated▫Side effects: drowsiness, weight gain, leg swelling, blurry
vision
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Itch/stiffness: UVA1• 3 different doses: low, medium, high
• Side effects: tanning, itching, headache
• Data are limited on future carcinogenesis risk
• May develop recurrent activity within the next 2 years▫Mean duration of disease before UVA1 was longer in
recurrent group, darker skin had less recurrent activity
• Patients that received UVA1 for varying diseases did not find a significant difference in response in whether they were light or dark skinned Sator, 2009; Kreuter, 2012; Vasquez, 2014;
Jacobe, 2008
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Itch/Stiffness: Ultraviolet Light Therapy
•PUVA▫Side effects: SKIN CANCER! Aging, burning,
itching, nausea, tanning, eye damage
•Narrowband UVB
•Broadband UVA
Kerscher, 1996; Pavlotsky, 2013; Brownell, 2007; Newland, 2012; El-Mofty M, 2000
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Rituximab•Improvement in skin score, lung fibrosis, calcinosis, itching•Rituximab: antibody against a molecule on the surface of
most B cells, a component of our immune system•Infusion (get it intravenously)
•Trials: ▫Currently recruiting for patients with lung disease for
randomized controlled trial in UK▫Trial for patients with pulmonary arterial hypertension (many
locations in the states)
Jordan S, 2014; Bosello SL 2015; Smith 2013; Giuggioli D, 2015
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Raynaud’s
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What is Raynaud phenomenon?• Defect in the blood vessels, cannot regulate temperature in the skin
• Episodic reduction in blood supply
• Most often on fingers but can also affect toes, feet, ears, nose
• Turns white in the cold, then turns blue/red when rewarming
• Can last from minutes to hours
• Medicines can make it worse so discuss with your doctor if you are on:▫ Beta blockers, clonidine, ergotamine (migraines), bromocriptine (Parkinson’s),
imipramine, OCPs
• If possible, avoid decongestants, amphetamines, diet pills, methylphenidate (treatment for ADD)
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TIPS for Raynaud’s: Prevention!• Minimize cold exposure
▫ Outdoor weather▫ Air conditioning▫ Refrigerator or freezer▫ Shopping/grocery stores▫ Cold beverages – Koozie/hugger
• Keeping warm: entire body and extremities▫ Gloves/mittens, hats, earmuffs, heavy socks –
have them in car, at work, at home▫ Layered clothing ▫ Cotton gets cold when wet! Avoid if you are going to break a sweat!
For exercise outdoors: synthetics, capelene, smart wool▫ Electric heaters, electric blankets and comforters, or heating pad/hot water
bottle ▫ Warm bath/shower▫ Space heaters at work, car warmers, hand warmers, warm hair dryer
• NO SMOKING!!!
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Digital ulcers• Due to poor blood flow, micro-trauma to
fingers, worse when skin is stiff
• Often on back side of hands in diffuse scleroderma, tips of finger in limited
• Seek physician involvement early if you have never had one and then develop!
• Recent study of > 2000 patients in Europe showed 60% patients had digital ulcers currently and ALL patients had history of ulcers
Denton, 2012
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Digital ulcers• Bosentan is medication that can decrease
amount of new ulcers but does not help healing of current ulcers
• If severe, injection of anesthesia helps open up the blood vessels
• Botox can also help: expensive, lack of good clinical trials
• Localized digital sympathectomy performed by hand surgeons
• If you develop blue/black digits, go to ERDenton, 2012
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Tips for digital ulcers!• Avoid infection!
▫ If red, warm, more tender than normal, pus—go see physician!▫ Talk to your doctor about need for bleach baths as preventative measures
if your ulcers frequently get infected: Bleach bath: For a 1/2 full tub, use 1/4 cup of bleach. Use a measuring cup for ¼ or ½ cup, NOT ¼ of cap!! Use household bleach, not concentrated bleach. Soak affected areas of skin for about 10 minutes. Do not submerge the head. Rinse off and gently pat dry with a towel
• Make sure your shoes fit properly, not too tight• Be honest about your pain: pain constricts blood vessels, making
ulcers worse• Take care of your overall health: heart health, no smoking, decrease
stress
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Cuticles
•Do NOT cut cuticles•Keep moisturized (see dry skin section)•Do NOT pick!
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Calcinosis• Calcium deposits in the skin• Firm, can be painful• See in areas of repeated trauma: fingers, hands, elbows, knees, shins
• Minocycline, colchicine, diltiazem
• Sodium thiosulfate: treatment for calciphylaxis given intravenously • Early case reports on intralesional or topical
• Lithotripsy: improved size, pain• Surgical removal: discrete spots
• 361 trials for calcinosis (most for other things besides scleroderma) on clinicaltrials.gov but hopefully something will come soon
Sultan-Bichat N, 2012
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Calcinosis TIPS!
•“Adequate” treatment of disease•Don’t try to squeeze out the material•Avoid trauma to area•Avoid infection as much as possible•Get involved with research if possible—still TOO much to
learn!▫Observational study at Rutgers
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Do I need a dermatologist?•YES!!•Rheumatologists often manage the disease in its
entirety including skin, lung, heart, kidney•Dermatology can be helpful to maximize skin comfort•If on immunosuppression medications:
▫Need a total body skin examination yearly
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Empower yourself!
•You already are empowered, you are HERE!•Get involved!•Research---there is SO much we still do not know•Face Off for Scleroderma campaign on social media•Blogger from Highland Park, Lisa,
www.comfortableinmythickskin.com
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References• Bosello SL, Semin Arthritis Rheum
2015;44(4):428.• Brownell I, Derm Online J 2007;13(1).• Cunningham BB et al. JAAD 1998;39(2 Pt
1):211.• Denton CP et al, Ann Rheum Dis 2012;71:718.• Dinsdale G, et al. Rheumatology
2014;53:1422.• Giuggioli, D, et al. Autoimmunity Reviews
2015;14(11):1972.• Halachmi S, et al. Lasers Med Sci
2014;29:137-40.• Hetzer S, et al. Eur J Medical Research
2014;19:2.• Jordan S, et al. Ann Rheum Dis 2014• Kreuter A, et al. JAAD 2012;67(6):1157-62.• Kroft EBM, et al. Am J Clin Dermatol
2009;10(3):181.
• Nymann P, et al. Br J Dermatol 2009;160:1237-41.
• Nymann P, et al. J Eur Acad Dermatol Venereol 2010;24:143-6.
• Pavlotsky F, et al. Photoderm, Photoimm, Photomed 2013;29(5):247.
• Pope E, et al. Dermatology 2011;223:363.• Tanghetti EA. Lasers in Surgery and
Medicine 2012; 44:97-102.• Sator PG, et al. JAAD 2009;60(5):786• Smith V, et al. J Rheumatol 2013;40(1):52.• Sultan-Bichat N, et al. JAAD
2012;66(3):424.• Uppal L, J Hand Surg Eur Vol
2014;39(8):876.• Vasquez R, et al. JAAD 2014;3:481
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Thank you!Questions ????