10303 PUST Pustular Psoriasis JUNE 12_Layout 4 22/08/2012 12:48 Page 1 Pustular Psoriasis A positive approach to psoriasis and psoriatic arthritis 10303 PUST Pustular Psoriasis JUNE 12_Layout 4 22/08/2012 12:48 Page 2 What are the aims of this leaflet? This leaflet has been written to help you understand more about different types of pustular psoriasis, including treatments. What is psoriasis? Psoriasis (sor-i’ah-sis) is a long-term (chronic) scaling disease of the skin, which affects 2% to 3% of the UK population. IT IS NOT CONTAGIOUS. It appears as red, raised, scaly patches known as plaques. Any part of the skin surface may be involved, but the plaques most commonly appear on the elbows, knees and scalp. It can be itchy, but is not usually painful. Nail changes, including pitting and ridging, are present in 40% to 50% of people with psoriasis and 10% to 20% of people with psoriasis will develop psoriatic arthritis. There are many different types of psoriasis, including chronic plaque psoriasis, and there are also many types of pustular psoriasis (see below), guttate psoriasis, scalp psoriasis, flexural psoriasis, napkin psoriasis, nail psoriasis and erythrodermic psoriasis – see our What is Psoriasis? leaflet. What happens? Normally a skin cell matures in 21 to 28 days and during this time it travels to the surface, where it is lost in a constant invisible shedding of dead cells. In patches of psoriasis the turnover of skin cells is much faster, around 4 to 7 days, and this means that even live cells can reach the surface and accumulate with dead cells. The extent of psoriasis and how it affects an individual varies from Plaque psoriasis person to person. Some may be 2 10303 PUST Pustular Psoriasis JUNE 12_Layout 4 22/08/2012 12:48 Page 3 mildly affected, with a tiny patch hidden away on an elbow which does not bother them, while others may have large visible areas of skin involved that significantly affect daily life and relationships. This process is the same wherever it occurs on the body. Pustular psoriasis tends not to look like plaque psoriasis, although plaque and pustular psoriasis can coexist or one may follow the other. The main distinguishing feature of pustular psoriasis is the appearance of pus spots surrounded by red skin. This doesn’t mean that there is any infection present. The spots simply show that the skin has been invaded by the same white blood cells that would be seen if there were to be an infection present. It can be triggered by some internal medications, irritating topical agents, Pustular psoriasis u l t r a v i o l e t l i g h t o v e rd o s e s , pregnancy, systemic steroids (especially sudden withdrawal of systemic or topical steroids), infections, perspiration or emotional stress. Generalised pustular psoriasis Generalised pustular psoriasis is a rarer form of the condition, and especially rare in children. It can be abrupt, and triggered by an infection, pregnancy, low thyroid activity or any of a number of different drugs. It can cause fever, chills, severe itching, rapid pulse rate, exhaustion, anaemia, weight loss, muscle weakness and/or joint pain. Sometimes these attacks are followed by milder outbreaks of psoriasis. With this type, the pustules occur all over the body – patients can often 3 © Bernard Cohen MD, Dermatlas http://www.dermatlas.org Pustular psoriasis 10303 PUST Pustular Psoriasis JUNE 12_Layout 4 22/08/2012 12:48 Page 4 become quite unwell and, as such, generalised pustular psoriasis often requires hospital in-stay treatment. Please do note that this is a rare form of psoriasis. Another form of pustular psoriasis, palmer-plantar pustulosis, causes pustules on the palms and soles of the feet. It tends to occur in people between the ages of 20 and 60, and is more common in people who smoke. Infection and stress are suspected trigger factors. PPP is normally recognisable by large yellow pustules up to 5cm in diameter in fleshy areas of hands and feet, such as the base of the thumb and the sides of the heels. It may PPP be painful. The pustules then turn brown and drop off or peel. PPP is usually cyclical with new crops of pustules followed by periods of low activity. This form of psoriasis affects approximately 5% of people with psoriasis. It tends to go in cycles of: ● erythema (reddening of the skin) followed by ● formation of pustules; and ● scaling of the skin. Topical therapies are usually a first-line treatment. PUVA and methotrexate are also sometimes used to clear the attack. However this type of psoriasis can be a little more difficult to manage. In most episodes of pustular psoriasis these will last for a few weeks then disappear or return to erythrodermic psoriasis. 4 © Shahbaz A Janjua MD, Dermatlas http://www.dermatlas.org Palmer-plantar pustulosis (PPP) 10303 PUST Pustular Psoriasis JUNE 12_Layout 4 22/08/2012 12:48 Page 5 Acrodermatitis continua of Hallopeau © Shahbaz A Janjua MD, Dermatlas http://www.dermatlas.org Another rare type of palmar-plantar pustular psoriasis, acrodermatitis continua of Hallopeau is characterised by skin lesions on the ends of the fingers and sometimes on the toes. The eruption occasionally starts after local trauma. Often the lesions are painful and disabling, with the nails often deformed, and bone changes may occur. This condition is quite hard to treat satisfactorily. Treatment Pustular psoriasis and PPP are usually treated with topical treatments. Sometimes topical steroids under thin dressings are prescribed. Pustular psoriasis can be stubborn to treat so sometimes other treatment regimes typically used for plaque psoriasis may be tried. Although not completely proven, for those who smoke, quitting appears to reduce the number of pustules as well as significantly reducing the erythema (redness) and desquamation (shedding of skin). The main aims of the treatment of generalised pustular psoriasis are to restore the skin’s barrier function, prevent further loss of fluid, stabilise the body’s temperature and restore the skin’s chemical balance. Imbalances, which can occur, might put added strain on the heart and kidneys, especially in older people. Because of possible complications with this form of psoriasis, if you’re affected you should seek medical care immediately. The likelihood of hospitalisation for a short period of time depends on the severity of the outbreak. When hospitalised, bed rest, mild sedation, bland topical therapy, rehydration and avoiding excessive heat loss can improve the situation. It is important to remove as many of the potential trigger factors as possible. In some cases antibiotics are prescribed just in case an infection is also present. In severe cases, where the patient has become exhausted, other medications may be needed. Methotrexate is the most common treatment for generalised pustular psoriasis. Ciclosporin is also used if your doctor 5 10303 PUST Pustular Psoriasis JUNE 12_Layout 4 22/08/2012 12:48 Page 6 needs to control the symptoms quickly. Oral steroids are often prescribed for those who do not respond to other forms of treatment or who have become very ill, but their use is very controversial. PUVA (the photsensitising drug psoralen plus UVA light) may also be used in the treatment of this condition after it has subsided. Acrodermatitis continua of Hallopeau tends to be resistant to both topical and systemic treatments for psoriasis, so combinations of therapy may be tried. Most episodes of pustular psoriasis will last for a few weeks then disappear or remit to erythrodermic psoriasis. Summary ● Pustular psoriasis is a rare form of psoriasis ● Severe cases need urgent referral ● Be aware of the trigger factors ● PPP is more common in people who smoke ● It tends to go in cycles ● Where pustules exist they may not necessarily be infectious ● Like psoriasis, the pustular version is not contagious. Always consult your doctor or healthcare provider. About this information This material was produced by PAPAA. Please be aware that treatments and research is ongoing. References and sources of evidence for this leaflet are available upon request or can be found on our website. For the latest information or any amendments to this material please contact us or visit our website. The site contains information on treatments and includes patient experiences and case histories. Original text written by David and Julie Chandler 1996. Reviewed/revised April 2004, June 2009, March 2011. 6 10303 PUST Pustular Psoriasis JUNE 12_Layout 4 22/08/2012 12:48 Page 7 This edition reviewed and revised by Dr Jennifer Crawley, Clinical Fellow in Medical Dermatology, St John’s Institute of Dermatology, London March 2012. A lay and peer review panel has provided key feedback on this leaflet. The panel includes people with or affected by psoriasis and/or psoriatic arthritis. Published: June 2012 Review date: March 2014 © PAPAA The Information Standard scheme was developed by the Department of Health to help the public identify trustworthy health and social care information easily. At the heart of the scheme is the standard itself – a set of criteria that defines good quality health or social care information and the methods needed to produce it. To achieve the standard, organisations have to show that their processes and systems produce information that is: ● accurate ● evidence-based ● impartial ● accessible ● balanced ● well-written. The assessment of information producers is provided by independent certification bodies accredited by The United Kingdom Accreditation Service (UKAS). Organisations that meet The Standard can place the quality mark on their information materials and their website - a reliable symbol of quality and assurance. 7 10303 PUST Pustular Psoriasis JUNE 12_Layout 4 22/08/2012 12:48 Page 8 ® The charity for people with 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 a principal source of information and educational material for people with psoriasis and psoriatic arthritis in the UK. PAPAA supports both patients and professionals by providing material that can be trusted (evidencebased), which has been approved and contains no bias or agendas. PAPAA provides positive advice that enables people to be involved, as they move through their healthcare journey, in an informed way which is appropriate for their needs and any changing circumstances. Contact: PAPAA PO Box 111 St Albans Herts AL2 3JQ Tel: 01923 672837 Fax: 01923 682606 Email: [email protected] www.papaa.org ISBN 978-1-906143-19-0 9 781906 143190 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 PUST/06/12
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