When it comes to dermatological or skin issues in PWS, the first things that may come to mind are sensitivity to the sun due to fair skin, and skin picking. However, there are a variety of additional skin-related concerns for individuals with PWS. Data from the Dermatology Survey in the Global PWS Registry shows that individuals with PWS are more likely to have seen a dermatologist as they get older. By 15 years of age, 28% of registry participants have seen a dermatologist, and that number rises to 45% by age 21.
Other than skin picking, the most commonly reported skin related issues in the Registry are eczema (9%), cellulitis (9%), and edema or swelling due to excess fluid (7%). Eczema is an inflammation of the skin, resulting in patches that are dry, scaly, and itchy. Eczema rashes can vary in severity. Although there is no cure for eczema, treatments are designed to help reduce inflammation and itchiness. Unfortunately, scratching due to eczema can led to open wounds and scabs that can promote skin picking, and persistent skin picking increases the risk of skin infections such as cellulitis.
Cellulitis is a skin infection caused by a bacterial infection. Areas of cellulitis may be red, swollen, tender, warm to the touch, and may expand or change over time. Symptoms may also be accompanied by a fever. Risk factors for cellulitis include injuries to the skin, skin conditions such as eczema and obesity, all of which suggest that individuals with PWS may be at higher risk to cellulitis than the general population. Although the incidence of cellulitis in the general population is not well defined, a 2006 paper in the journal Epidemiology and Infection reported cellulitis infection rates of 1.5%-3.5%, depending upon age group. This is much lower than the cellulitis infection rates reported for individuals with PWS within the Registry. Within the Registry more than 20% of participants have had a cellulitis infection by age 21. Notably, 29% of the cellulitis infections reported in the Registry were serious enough to require hospitalization. This suggests individuals with PWS should be monitored for cellulitis, and families should be quick to seek medical care if signs of infection appear.
You can find more information about the Global PWS Registry on our website, or by visiting the registry at www.pwsregistry.org, where you can join or update your surveys. You may also email our registry administrator here.