Skin Care in SclerodermaLouise ParkerLead Nurse – Connective Tissue Disease

Background

• Rare

• Autoimmune connective tissue disease

• Collagen overproduction

• Skin tightening

• Internal organ dysfunction

The spectrum

Vascular

Scleroderma

Fibrotic

Raynaud’s phenomenon

Localised

Inflammatory,Vascular & Fibrotic features

Types of Scleroderma

Localised

Morphoealocalised andgeneralised

Linear sclerodermaEn coup de sabre

Systemic

Limited

Diffuse

Scleroderma sine scleroderma

Overlap

The skin

What does it do for us?

• Our personal barrier to the outside world

• Packed full of sensors to protect from injury and exposure to the elements

• Although it is our protection it is also full of bugs!

• Our biggest organ and accounts for around 15% of our total body weight

Common Problems in scleroderma

• Itchy/dry skin

• Digital ulcers

• Telangiectasia/pigmentation changes/camouflage

• Calcinosis

Dry skin & Itchiness

• The overproduction ofcollagen being laid down inthe skin means that the skin becomes tight, stiff and dry

• This can also mean manypatients will also experienceItchiness

• Immunosuppression?

Dry/itchy skin

• Emollients - NOT SOAP for washing/bathing Aveeno, Hydromol, Epaderm, Doublebase

• Soap is drying on the skin due the ingredients used – various chemicals, detergents and preservatives

• Additives that make soap ‘lather’ can also be drying and irritate the skin

• No shower gel, liquid hand soap, ‘cream’ bath or shower creams or alcohol gels if possible

Dry/itchy skin treatments - topical

• Ointments ‘Greasy’Doublebase, Dermol, Diprobase, E45

• Cream Flexitol, Doublebase, Diprobase

• Gel ‘Lighter’Doublebase/Dayleve gel

Other dry/itchy skin treatments

• Specific creams 1% menthol, Balneum, E45 anti-itch

• Antihistamines OTC - Benadryl, PiritonPrescription - Montelukast, Citirizine

• Practical Hand waxing/protection/sunscreen

Digital Ulceration• Classical outward sign of scleroderma• Partial or full thickness skin loss often over fingers or

toes but also over pressure points. • Combination of Raynaud’s and skin changes in

Scleroderma can lead to tissue breakdown resulting in a digital ulcer.

• Around 40% of scleroderma patients suffer with digital ulceration

Manifestations

• Some patients with persistent ulcers may develop gangrene

• Infection is common if healing time is prolonged

• Often slow to heal because of poor circulation and tight skin

• Huge impact on quality of life – pain, activities of daily living, washing, grooming, preparing food

Digital ulcer treatments

• Medication review – optimisation of Raynaud’s treatment & pain management

• Vasodilators, oral & intravenous

• Antibiotics

• Expert wound management

• Patient concordance

Digital ulcer treatments

• Antioxidant treatment – Vitamin E and C

• Evening Primrose Oil

• Sildenafil + Iloprost

• Bosentan

• Innovative therapy – from PAH

Telangiectasia

• Dilated superficial blood vessels – face, chest, hands

• A classical outward sign of scleroderma

Telangiectasia

• Usually harmless but can affect body image as they can be difficult to cover

• Troublesome if they bleed internally (‘GAVE’ or ‘watermelon stomach’) as this can cause anaemia but treatable with laser

• Two main approaches can be helpful

Telangiectasia treatments

• Laser To breakdown the vessels making them look less obvious – dermatology units offer this

• Cosmetic camouflageWax based, waterproof makeup – excellent coverage Charity ‘Changing Faces’ by self referral

• OTC Make upDermablend (Vichy) MAC Cosmetics

Calcinosis

• This is either hard lumps of a ‘chalky’ like substance that works its way out through the layers of the skin

OR• Rises to the surface of the skin in a ‘toothpaste’

like consistency which then leaks out from the wound

• Extremely painful, often leads to infection and can precede an ulcer

Calcinosis

• Often appears over pressure points - fingers, elbows, knees but also over buttocks and abdomen

• Can lay down in the skin as large plaques which can cause functional disability as well as pain and distress

• Often gets ‘picked at’ by patients!

Calcinosis treatments

• Poorly understood pathology so usually treated conservatively

• Expert wound management

• Antibiotics if infected +/- vasodilators

• Surgery to remove large areas or in places that affect function/ROM – but can come back

Conclusion

• Lots of practical and self help measures can be useful – change of routine/making time/consistency

• Local GP and local pharmacists can offer advice

• Referral on to a Dermatologist

• Specialist / larger units will often work with several departments and other specialists who can help

Remember…

• Everyone is different

• Symptoms vary from person to person

• Can occur in different stages in varying degrees of severity

• What may work for you, may not for someone else

Any Questions