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A positive approach to psoriasis and psoriatic arthritis Pustular Psoriasis

Pustular Psoriasis - Psoriasis and Psoriatic Arthritis ... · Pustular psoriasis looks different to plaque psoriasis, although plaque and pustular psoriasis can coexist or one may

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Page 1: Pustular Psoriasis - Psoriasis and Psoriatic Arthritis ... · Pustular psoriasis looks different to plaque psoriasis, although plaque and pustular psoriasis can coexist or one may

A positive approach

to psoriasis and

psoriatic arthritis

Pustular Psoriasis

Page 2: Pustular Psoriasis - Psoriasis and Psoriatic Arthritis ... · Pustular psoriasis looks different to plaque psoriasis, although plaque and pustular psoriasis can coexist or one may

What are the aims of this leaflet?This leaflet has been written to help you understand more aboutdifferent types of pustular psoriasis, as well as suitabletreatments.

What is psoriasis?Psoriasis (sor-i’ah-sis) is a long-term (chronic) scaling diseaseof the skin, which affects around 2% of the UK population. It isnot contagious. It appears as red, raised, scaly patches knownas plaques. Any part of the skin surface may be involved, butthe plaques most commonly appear on the elbows, knees andscalp. It can be itchy, but is not usually painful. Nail changes,including pitting and ridging, are present in 40% to 50% ofpeople with psoriasis and around 30% of people with psoriasiswill develop psoriatic arthritis. There are many different types ofpsoriasis, including chronic plaque psoriasis, types of pustularpsoriasis (all of which are described in this leaflet), guttatepsoriasis, scalp psoriasis, flexural psoriasis, napkin psoriasis,nail psoriasis and erythrodermic psoriasis (a rare, seriouscondition which can affect the whole body) – see our What is

Psoriasis?, Scalp Psoriasis and Nail Psoriasis leaflets.

What happens?Normally a skin cell matures in 21 to 28 days and during thistime it travels to the surface, where it islost in a constant invisibleshedding of dead cel ls . Inpatches of psor iasis theturnover of skin cells is muchfaster, around 4 to 7 days, andthis means that even live cellscan reach the surface andaccumulate with dead cells.The extent of psoriasis, andhow it affects an individual,varies from person to person.Some may be mildly affected, witha tiny patch hidden away which doesnot bother them, while others may have large visible areas ofskin involved that significantly affect daily life and relationships.The process is the same wherever it occurs on the body.

Pustular psoriasisPustular psoriasis looks different to plaque psoriasis, althoughplaque and pustular psoriasis can coexist or one may follow theother. The main distinguishing feature of pustular psoriasis is theappearance of pus spots surrounded by or on top of areas ofred skin. This does not mean there is infection present. The

Plaque psoriasis

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spots simply show that the skin hasbeen invaded by white bloodcells. The person is not infecteda n d n e i t h e r a r e t h e ycontagious in any way. Pustular psoriasis flare-ups

c a n b e t r i g g e r e d b y some medicines, irritatingsubstances on the skin,ultraviolet light overdoses,pregnancy, systemic steroids(especially sudden withdrawal oftablet or h igh-potency topicalsteroids), infections or emotional stress.

Generalised pustular psoriasisGeneralised pustular psoriasis is a rarer and more serious formof the condition. It is particularly rare in children. It can occurabruptly, sometimes precipitated by the triggers mentionedabove, and it can make the person feel quite unwell with fever,shivers, intense itching, rapid pulse, exhaustion, headache,nausea, muscle weakness and/or joint pain. In this condition,the pustules, redness and skin peeling can occur all over thebody and, as such, people are normally admitted to hospital fortreatment under the care of a dermatologist until it settles down.The skin can be very sore and fragile. Sometimes these attacksare followed by milder outbreaks of psoriasis. Please do notethat this is a rare form of the condition.

Palmar-plantar pustulosis (PPP)Palmar-plantar pustulosis (PPP – also sometimes spelled aspalmoplantar psoriasis) is a localised form of psoriasis whichaffects the palms and/or soles of the feet. Evidence suggeststhat it tends to occur in people between the ages of 20 and 60,and is more common in people who smoke. Infection and stressare suspected trigger factors. PPP is normally recognisable bylarge yellow pustules up to 5mm in diameter in the fleshy areasof hands and feet, such as the base of the thumb and the sidesof the heels. It may be painful. The pustules dry up and turn abrown colour and then gradually diminish. PPP is usually cyclicalthough, with new crops of pustules being followed by periods oflow activity. This form of psoriasis affects approximately 5% ofpeople with psoriasis. It tends to go in cycles of: erythema(reddening of the skin) followed by formation of pustules andthen scaling of the skin. Sometimes the skin can crack, whichcan cause discomfort, and occasionally there is a degree offunctional disability.

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Pustular psoriasis

© Bernard Cohen MD, Dermatlas http://www.dermatlas.org

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Acrodermatitis continua ofHallopeauAcrodermatitis continua of Hallopeau is another rare type ofpalmar-plantar pustular psoriasis. It ischaracterised by skin lesions on theends of the fingers, including thenai ls which can somet imesbecome completely destroyed,and sometimes on the toes.The eruption sometimes startsa f te r loca l i sed phys ica ltrauma. Often the lesions arepainful and disabling, with thenails deformed, and bonechanges may occur. Thiscondition is quite hard to treatsatisfactorily.

TreatmentThese conditions are not common and are difficult to treat, sowhether the pustular psoriasis is localised or generalised,treatment should be overseen by a dermatologist.

Pustular psoriasisIf you are admitted to hospital with generalised pustularpsoriasis, the main aim of treatment is to restore the skin’sbarrier function, prevent further loss of fluid, stabilise the body’stemperature and restore the skin’s chemical balance.Imbalances, which can occur, might put added strain on theheart and kidneys, especially in older people. Because ofpossible complications with this form of psoriasis, if you’reaffected you should seek medical care immediately. Thelikelihood of hospitalisation for a short period of time dependson the severity of the outbreak. When in hospital, bed rest, blandemollient therapy, rehydration and avoiding excessive heat losscan improve the situation. It is important to remove as many ofthe potential trigger factors as possible, for example suspectmedicines. In severe cases, where the patient has becomeexhausted, other medications may be needed.To control pustular psoriasis for the longer term, a tablet orinjection treatment is usually required.Methotrexate is the most common treatment; ciclosporin is

also used if your doctor needs to control the symptoms quickly.Oral steroids are often prescribed for those who do not respondto other forms of treatment or who have become very ill, but theiruse would be for a brief period only. PUVA (the photosensitisingdrug psoralen plus UVA light) may also be used once the

PPP

© Shahbaz A Janjua MD, Dermatlas http://www.dermatlas.org

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condition has settled down. See our Psoriasis and

Phototherapy leaflet to find out more about PUVA. In someinstances biologic injection treatments may be used, but theseare not licensed for pustular psoriasis.

Palmar-plantar pustulosis (PPP)treatment optionsThis type of psoriasis can be particularly difficult to treat. First-line treatment usually involves topical therapies, such aspotent or super-potent steroids, being applied to the palmsand/or soles. Sometimes creams containing coal tar or salicylicacid may be prescribed to help with scaling. The regularapplication of an emollient can help prevent skin from crackingand keep the palms and soles more supple. It may be beneficialto wear cotton gloves for certain periods during the day, toprevent the treatments rubbing off and to allow the person tocontinue to use their hands.Frequent use of topical therapies on the hands and feet can

be impractical for most people as it makes working and daily chores difficult. For this reason, localised light treatment(PUVA) or tablet treatments such as methotrexate are oftenadvised.Although not completely proven, for those who smoke,

quitting appears to help reduce the instances and severity ofpalmar-plantar psoriasis.Acrodermatitis continua of Hallopeau tends to be resistant to

both topical and systemic treatments for psoriasis, socombinations of therapy may be tried. Most episodes of pustularpsoriasis will last for a few weeks then disappear or remit toerythrodermic psoriasis.

Summary� Pustular psoriasis is a rare form of psoriasis

� Severe cases need urgent referral to a specialist andoften hospital admission

� PPP is more common in people who smoke

� PPP and pustular psoriasis flares tend to go in cycles

� The pustules are not an infection but a collection ofwhite cells

� Pustular psoriasis is not contagious.

If you have any views or comments about this information or anyof the material PAPAA produces, you can contact us via thedetails on the back page or online at www.papaa.org/user-feedback

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Useful contactsFor information about health matters in general and how toaccess services in the UK, the following websites providenational and local information.

� NHS Choices (England): www.nhs.uk� NHS 24 (Scotland): www.nhs24.com� Health in Wales: www.wales.nhs.uk� HSCNI Services (Northern Ireland):

http://online.hscni.net

These sites are the official sites for the National Health Serviceand provide links and signposting services to recognisedorganisations and charities.

References:Gelfand JM, Weinstein R, Porter SB, Neimann AL, Berlin JA,Margolis DJ. Prevalence and treatment of psoriasis in the United Kingdom: A population-based study. Arch Dermatol2005;141:1537-41.

Cohen MR, Reda DJ, Clegg DO. Baseline relationships between psoriasis and psoriatic arthritis: Analysis of 221patients with active psoriatic arthritis. Department of VeteransAffairs cooperative study group on seronegativespondyloarthropathies. J Rheumatol 1999;26:1752-6.

Alamanos Y, Voulgari PV, Drosos AA. Incidence and prevalenceof psoriatic arthritis: A systematic review. J Rheumatol2008;35:1354-8.

Ibrahim G, Waxman R, Helliwell PS. The prevalence of psoriaticarthritis in people with psoriasis. Arthritis Rheum 2009;61:1373-8.

Krueger JG, Bowcock A. Psoriasis pathophysiology: Currentconcepts of pathogenesis. Ann Rheum Dis 2005;64 Suppl2:ii30-6.

Reitamo S, Erkko P, Remitz A. Palmoplantar pustulosis. Eur JDermat 1992; 2: 311-4

Barker H. Pustular psoriasis. Dermat Clin 1984; 2: 455.

Rook’s Textbook of Dermatology, Eighth Edition. Eds: Burns T,Breathnach S, Cox N, Griffiths C. Wiley-Blackwell, Oxford.

Hagforsen E, Michalsson G. Palmoplantar Pustulosis –Improvement after cessation of smoking. Abstract Number 391.34th Annual ESDE Meeting. 9-11 September 2004, Vienna.Austria.

O’Doherty C J, Macintyre C. Palmoplantar pustulosis andsmoking. British Medical Journal Volume 291, 28 September1985.

The above list is not exhaustive. For further references used inthe production of this and other PAPAA information, contact usor go to: www.papaa.org/resources/references

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The Information Standard scheme was developed by theDepartment of Health to help the public identify trustworthyhealth and social care information easily. At the heart of thescheme is the standard itself – a set of criteria that definesgood quality health or social care information and themethods needed to produce it. To achieve the standard,organisations have to show that their processes and systemsproduce information that is:

� accurate � evidence-based

� impartial � accessible

� balanced � well-written.

The assessment of information producers is provided byindependent certification bodies accredited by The UnitedKingdom Accreditation Service (UKAS). Organisations thatmeet The Standard can place thequality mark on their informationmaterials and their website - areliable symbol of quality andassurance.

About this informationThis material was produced by PAPAA. Please be aware thatresearch and development of treatments is ongoing. For the latest information or any amendments to this material,please contact us or visit our website: www.papaa.org. The sitecontains information on treatments and includes patientexperiences and case histories. Original text written by David and Julie Chandler 1996.Reviewed/revised April 2004, June 2009, March 2011.A full review and revision was carried out in March 2012 by Dr Jennifer Crawley, clinical fellow in medical dermatology, StJohn’s Institute of Dermatology, London and by consultant nurseKarina Jackson, St John’s Institute of Dermatology, Guy’s and StThomas’s NHS Foundation Trust in November 2014.A peer review has been carried out by Dr Thivi Maruthappu MAMRCP, senior clinical fellow in medical dermatology, St John'sInstitute of Dermatology in February 2017. A lay review panel has provided key feedback on this leaflet. Thepanel includes people with or affected by psoriasis and/orpsoriatic arthritis.Published: April 2017Review date: October 2019© PAPAA

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The charity for peoplewith psoriasis and psoriatic arthritis

PAPAA, the single identity of the Psoriatic Arthropathy Alliance and the

Psoriasis Support Trust.

The organisation is independently funded and is aprincipal source of information and educationalmaterial for people with psoriasis and psoriatic

arthritis in the UK.

PAPAA supports both patients and professionals byproviding material that can be trusted (evidence-

based), which has been approved and contains nobias or agendas.

PAPAA provides positive advice that enablespeople to be involved, as they move through their healthcare journey, in an informed way which is appropriate for their needs and any

changing circumstances.

Psoriasis and Psoriatic Arthritis Alliance is a company limited by guarantee

registered in England and Wales No. 6074887

Registered Charity No. 1118192

Registered office: Acre House, 11-15 William Road, London, NW1 3ER

Contact:PAPAA

3 Horseshoe Business Park, Lye Lane, Bricket Wood, St Albans, Herts. AL2 3TA

Tel: 01923 672837Fax: 01923 682606

Email: [email protected]

www.papaa.org

®

PUST/04/17

9 781906 143190

ISBN 978-1-906143-19-0