Wells’ syndrome is a rare disease in which there are itchy, burning, red and inflamed areas that can occur anywhere on the skin; it is also called eosinophilic cellulitis. Fewer than 100 cases have been reported throughout the world.
What is Wells’ syndrome?
Wells’ syndrome is a rare disease which also goes by the name "eosinophilic cellulitis." In this disease, itchy, burning, red, and inflamed areas can form anywhere on the skin. The inflamed areas can look like a skin infection called cellulitis, but there is no true infection present.
Eosinophils are a type of white blood cell that helps fight off infections. If the body over-produces eosinophils or the eosinophils are over-active, they can cause inflammation that damages the skin.
How common is Wells’ syndrome?
Wells’ syndrome is very rare. Fewer than 100 cases have been reported worldwide. It usually affects adults, but has also been found in children. Men and women are equally affected.
What causes Wells’ syndrome?
The cause of Wells’ syndrome is not known but it is thought to possibly be an autoimmune disorder. In such diseases, a person’s immune system mistakenly views healthy body tissue as an invader and starts to attack and destroy it. Most cases are random, but the disease may run in some families.
Other possible triggers include the following:
Arthropod bites and stings, including mosquito bites
Giardiasis (an intestinal infection)
Reactions to certain drugs or metals
What are the symptoms of Wells’ syndrome?
Symptoms usually begin with an itchy or burning sensation in one or more areas of skin, followed by redness and swelling. These areas are often painful. Sometimes, several new blisters or nodules are the first sign of the condition. Other possible symptoms include ring-shaped patches or itchy welts.
Bodily symptoms such as asthma, joint pain, and fever are possible but usually do not occur.
Over time, the skin changes color to brown-red, then blue-gray, then greenish-gray. Within a matter of weeks to months, the patches usually heal without any lasting effect, although they may come back again at a later time.
How is Wells’ syndrome diagnosed?
Skin biopsy is the diagnostic method of choice. A complete blood count may show a high number of eosinophils.
Skin biopsy will show a high number of eosinophils in the skin and underlying tissue, along with patterns of inflammation that are consistent with Wells’ syndrome but not with other possible causes of inflammation such as allergy, parasites, dermatitis, insect bites, or Churg-Strauss syndrome.
How is Wells’ syndrome treated?
One usually effective treatment is prednisone, a systemic steroid. Systemic steroids are those that are swallowed or injected. They have an immunosuppressant effect, meaning that they keep the immune system from overreacting and attacking one’s own body.
Prednisone is a synthetic version of cortisol, the natural steroid produced by the adrenal glands. Taken by mouth, prednisone usually leads to improvement within several days, and symptoms can be gradually reduced over a period of about a month.
Other possible treatments include antifungals (such as griseofulvin) or antibiotics (such as dapsone).
In mild cases, a steroid cream (such as hydrocortisone) rubbed onto the affected areas may be all that is necessary for treatment.
How can Wells’ syndrome be prevented?
Since the cause of Wells’ syndrome has not been identified, it is not yet possible to determine ways to avoid it.
What is the prognosis for those with Wells’ syndrome?
The outlook for people with the Wells’ syndrome is very promising. Flare-ups usually clear within a matter of weeks to months, usually without any lasting scars. Sometimes, the affected areas may heal with alteration of the skin color, but this usually fades with time. The disease may reappear. In these recurring cases, it may take years to finally get under control.