Skin Complications in Scleroderma
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Skin Complications in
Scleroderma
Emily L Keimig, MS, MD
Clinical Instructor
Department of Dermatology
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Objectives
• To address the various cutaneous complications of scleroderma
• Discuss treatment options for these various complications
• Discuss gentle skin care
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Raynaud’s Phenomenon
• First described in 1862 as Raynaud’s Disease
• 40 years later, proposed that ‘phenomenon’ was more appropriate
– Multiple causes of vasospasm
• Today classified as primary or secondary Raynaud’s phenomenon
– Primary
– Secondary
Bakst R et al. Raynaud's phenomenon: pathogenesis and management. J Am Acad Dermatol 2008;59:633-53
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Primary Raynaud’s Phenomenon
• Younger age of onset
• Normal nail fold capillaries
– cuticles
• Negative or low titers of auto-antibodies
• All fingers symmetrically affected
• Minimal pain
Bakst R et al. Raynaud's phenomenon: pathogenesis and management. J Am Acad Dermatol 2008;59:633-53
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Secondary Raynaud’s Phenomenon
• Associated with autoimmune disease
• Onset >30 years of age
• Frequently attacks are painful
• Asymmetric finger involvement
• Nail fold capillary abnormalities
• Pits and ulcers on the finger tips
• Elevated auto-antibodies
Bakst R et al. Raynaud's phenomenon: pathogenesis and management. J Am Acad Dermatol 2008;59:633-53
N Engl J Med 360;19
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Digital Pits
Bolognia J et al. Dermatology, 3rd Edition
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Nail fold Capillary Changes
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Cause
• Reversible vasospasm
– Vessels have altered responsiveness to vasoconstrictive and vasodilatory stimuli
– Abnormal release of vasoconstricting and vasodilating molecules
– Overactive sympathetic nerve receptors
• Arterial damage
– Thickening of the walls of the arteries
– Obstruction or blockage of the blood vessel
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Raynaud’s Phenomenon
• Episodic attacks – Hands, feet, nose, ears – Minutes to hours
• Cold exposure • Emotional stimuli • Tri-color change
– White/Pale – Blue – Red
• Numbness • Re-warming
– Can be painful
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Lifestyle Modifications
• Minimize cold exposure – 65F
• Limit time outdoors – Insulated mittens
– Hand/foot warmers
• Dress warmly – Loose-fitting
– Layered clothing
• Thermostat – Few degrees higher
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Lifestyle Modifications
• Keep whole body warm
– NOT just hands and feet
• Keep gloves everywhere
– Kitchen
– Car
– Work
• Extra layers of clothing
– Air conditioning can trigger
• Space heater
• Car warmed up
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Lifestyle Modifications
• Smoking
– Stop
– Avoidance of secondhand
• Stress modification
– Social support
– Relaxation techniques
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Other Things to Avoid
• Cold preparations
– Containing sympathomimetics
– Ephedrine
• Caffeinated beverages
• Ergots (migraine medications)
• Smoking
• Epinephrine
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Treatment
• Re-warming
– Warm environment
– Local heat • Warm water
• Warm hair dryer
• Medical intervention
– Medical
– Surgical
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Medical Treatment
• Topical therapy
– Nitroglycerin paste
• Calcium channel blockers
• Sildenafil
• Losartan
• Botox injection
• Peripheral digital sympathectomy
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Ischemic Ulcerations
• Severe Raynaud’s Phenomenon
• Common complication
• Nail fold changes a risk factor
– Dilatation and dropout
• Due to decreased blood flow
– Leads to decreased oxygenation of skin
Alivernini S et al. Skin ulcers in systemic sclerosis: determinants of presence and predictive factors of healing. J Amer Acad Dermatol. 2009;60:426-35
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Prevention
• Avoidance of trauma
• Properly fitting shoes
– ½ inch between front of shoe and toes
– All toes should be extended
– Good heel support
• Avoid medications that cause vasoconstriction
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Treatment
• Therapies aimed at improving blood flow and controlling infection
• Wound care
– Hydrocolloid dressings
• Topical nitroglycerin
• Calcium channel blocker
• IV therapies
– Prostanoids
• Bosentan
• Peripheral digital sympathectomy
Alivernini S et al. Skin ulcers in systemic sclerosis: determinants of presence and predictive factors of healing. J Amer Acad Dermatol. 2009;60:426-35
Bakst R et al. Raynaud's phenomenon: pathogenesis and management. J Am Acad Dermatol 2008;59:633-53
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Calcinosis Cutis
• Deposits of calcium within the skin
• Result of local tissue damage or abnormalities
• Occurs in various conditions
• 25-40% of patients with limited cutaneous systemic sclerosis
• Local irritation, inflammation, ulceration
• Fingers, forearms, elbows
Reiter N et al. Calcinosis cutis part I: diagnostic pathway. J Amer Acad Dermatol. 2011;65-1-12.
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Calcinosis Cutis
Bolognia et al. Dermatology. 3rd edition
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Treatment Calcinosis Cutis
• Warfarin
• Bisphosphonates
• Antibiotics
– Minocycline
– Decreased inflammation
• Diltiazem
• IVIg
– Decreased inflammation and symptoms
• Surgical Excision
• CO2 laser destruction
Reiter N et al. Calcinosis cutis, part II: treatment options. J Amer Acad Dermatol. 2010;65:15-22
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Telangiectasia
• Widened blood vessels
• Hands and face
– But can be anywhere
• Laser treatments
– Target the vessels
– Tend to come back
• Make up to cover
– Dermablend Bolognia et al. Dermatology. 3rd edition
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Discoloration
• Diffuse hyperpigmentation
• Overlying pressure points
• ‘salt and pepper’ discoloration
• Increased pigmentation overlying veins
Bolognia et al. Dermatology 3rd edition.
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Mobility
• Physical therapy
• Yoga
• Maintaining physical activity
– Low impact exercise
– Stretch
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Dry Skin
• Very common
• Decreased sweat glands
• Decreased oil glands
• Itching
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Itching = Pruritus
• Almost half of all patients
• Significant impact on quality of life
– Affects ability to fall and stay asleep
– Affects ability to concentrate
– Can lead to anxiety and depression
– Affects daily activities
– Affects personal relationships
– Impacts social functions
Ghassan EB et al. Association of Pruritus with Quality of Life and Disability in Systemic Sclerosis. Arthritis Care & Research. 2010; 62: 1489-95
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Itching
• Can result from dry skin and irritation
• Emollients – After bathing
– Locks in moisture
• Gentle skin care
• Medical treatment – Anti-histamines
– Light treatments
– Prescription therapy
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Gentle Skin Care
• Bathe at MOST once daily
– Every other day is fine
• Short showers or baths
– 5-10 minutes
• Lukewarm showers
• Do not scrub vigorously
– No brushes, loofahs, sponges
– Gently lather the soap
• Pat the skin dry
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Gentle Skin Care
• Avoid rubbing alcohol
• Avoid perfumes
• Rub don’t scratch
• Soothing baths
– Oatmeal baths
– No bubble baths
• Cooling agents
– Sarna (camphor)
– Menthol
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Moisturizers
• Oil based
– Petrolatum
• Water in oil products
– Heavier creams as opposed to lotions
• Products containing lipids found in the stratum corneum
– Lipids, ceramides, fatty acids
• Avoidance of fragrances, dyes, perfumes
• Apply to entire body within 2-3 minutes of bathing
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References
• Bakst R et al. Raynaud's phenomenon: pathogenesis and management. J Am Acad Dermatol 2008;59:633-53
• Gabrielli A et al. Scleroderma. N Engl J Med. 2009;360:1989-2003.
• Ghassan EB et al. Association of Pruritus with Quality of Life and Disability in Systemic Sclerosis. Arthritis Care & Research. 2010; 62: 1489-95
• Alivernini S et al. Skin ulcers in systemic sclerosis: determinants of presence and predictive factors of healing. J Amer Acad Dermatol. 2009;60:426-35
• Reiter N et al. Calcinosis cutis part I: diagnostic pathway. J Amer Acad Dermatol. 2011;65-1-12.
• Reiter N et al. Calcinosis cutis, part II: treatment options. J Amer Acad Dermatol. 2010;65:15-22