Skin Complications in

Scleroderma

Emily L Keimig, MS, MD

Clinical Instructor

Department of Dermatology

Objectives

• To address the various cutaneous complications of scleroderma

• Discuss treatment options for these various complications

• Discuss gentle skin care

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

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

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

Digital Pits

Bolognia J et al. Dermatology, 3rd Edition

Nail fold Capillary Changes

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

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

Lifestyle Modifications

• Minimize cold exposure – 65F

• Limit time outdoors – Insulated mittens

– Hand/foot warmers

• Dress warmly – Loose-fitting

– Layered clothing

• Thermostat – Few degrees higher

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

Lifestyle Modifications

• Smoking

– Stop

– Avoidance of secondhand

• Stress modification

– Social support

– Relaxation techniques

Other Things to Avoid

• Cold preparations

– Containing sympathomimetics

– Ephedrine

• Caffeinated beverages

• Ergots (migraine medications)

• Smoking

• Epinephrine

Treatment

• Re-warming

– Warm environment

– Local heat • Warm water

• Warm hair dryer

• Medical intervention

– Medical

– Surgical

Medical Treatment

• Topical therapy

– Nitroglycerin paste

• Calcium channel blockers

• Sildenafil

• Losartan

• Botox injection

• Peripheral digital sympathectomy

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

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

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

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.

Calcinosis Cutis

Bolognia et al. Dermatology. 3rd edition

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

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

Discoloration

• Diffuse hyperpigmentation

• Overlying pressure points

• ‘salt and pepper’ discoloration

• Increased pigmentation overlying veins

Bolognia et al. Dermatology 3rd edition.

Mobility

• Physical therapy

• Yoga

• Maintaining physical activity

– Low impact exercise

– Stretch

Dry Skin

• Very common

• Decreased sweat glands

• Decreased oil glands

• Itching

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

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

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

Gentle Skin Care

• Avoid rubbing alcohol

• Avoid perfumes

• Rub don’t scratch

• Soothing baths

– Oatmeal baths

– No bubble baths

• Cooling agents

– Sarna (camphor)

– Menthol

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

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