Management of the Skin in Scleroderma

Lauren Graham, MD, PhDClinical InstructorNorthwestern Department of DermatologyScleroderma Patient Education ConferenceOctober 10, 2015

No disclosures•Off-label uses of medications will be discussed today•Always discuss with your personal physician

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

Functions of Skin

•Protection: ▫Keeps the insides in, the outside

out! ▫Temperature regulation

•Cosmetic: ▫Our first impression to the world

Telangiectasias

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

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

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

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

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

•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

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

Dry skin/itch

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

• 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

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”

•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!

• 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!

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

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

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

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

Raynaud’s

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)

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!!!

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

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

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

Cuticles

•Do NOT cut cuticles•Keep moisturized (see dry skin section)•Do NOT pick!

DermNetNZ.org

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

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

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

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

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

Thank you!Questions ????