What is Wells Syndrome?
Wells syndrome, first identified in 1971, is a rare skin condition with unknown causes. It tends to be harmless but may come and go over time. This syndrome primarily affects adults but can also appear in individuals of any age. In some instances, this skin condition has been noticed in certain families, but usually, it occurs randomly without a clear pattern.
What Causes Wells Syndrome?
The exact cause of Wells syndrome isn’t known. It’s thought to possibly arise from an improper reaction from a type of white blood cell called an eosinophil, in response to a large range of triggers. This issue might be because the systems that control eosinophils aren’t functioning properly.
Different factors can trigger Wells syndrome. Some of the common ones include insect bites, certain medications, allergic skin reactions, a type of bone marrow disorder known as a myeloproliferative disorder, and various infections. These infections can include ones caused by fungi (dermatophytes), viruses, or a specific type of parasite (Toxocara canis).
Risk Factors and Frequency for Wells Syndrome
Wells syndrome is a rare skin condition with less than 200 documented cases reported so far. Although it’s primarily seen in adults, children can also develop this condition.
Signs and Symptoms of Wells Syndrome
In 1971, a condition was described by Wells as “recurrent granulomatous dermatitis with eosinophilia.” Later, it was renamed as “eosinophilic cellulitis” or often referred to as “Wells syndrome”.
Wells syndrome is a disease that begins suddenly. Its most common symptoms are large, well-defined red and swollen patches on the skin, which are often encapsulated with vesicles or blisters. These patches usually appear on the trunk and limbs. Before these develop, a person may often experience itching or burning sensations. Other general symptoms are uncommon, but a person might encounter a mild fever coupled with skin problems.
Over the next several days, the patches may expand, forming a ring-like shape. The center of these rings usually starts to heal, while the edges turn purple. The inflammatory signs typically lessen in about 10 days, while the skin patches harden. Complete healing usually occurs within 4 to 6 weeks.
Recurrences of Wells syndrome are common, and these can appear on varied locations on the body. The time between recurrences is inconsistent and may range from a few months to several years. Overall, the long-term recovery and health outcome is generally good for people with this syndrome.
Testing for Wells Syndrome
In simpler terms, around 50% of patients show an increased number of a certain type of white blood cell, called eosinophils, in their blood during the initial stage of the illness. This condition is referred to as peripheral blood eosinophilia.
Treatment Options for Wells Syndrome
The treatment for Wells syndrome, a rare skin disorder, isn’t standardized. The main treatments are typically steroids and a medication called dapsone, along with addressing any underlying conditions that might be triggering the syndrome.
Corticosteroids are often used to treat many types of skin disorders, including Wells syndrome. They can help reduce the duration and severity of relapses in about 10% of cases. Dosages vary, and they’re commonly reduced over time. Sometimes, flares can occur when doses are being reduced, which can sometimes lead to a reliance on steroids. If the Wells syndrome is superficial, applying steroids to the skin can be an alternative to oral medication, although the results vary.
Dapsone is another common treatment for Wells syndrome. It can help reduce the duration of relapses. The optimal treatment length isn’t clearly defined but can be several months if there aren’t any side effects that require stopping the treatment. Dapsone can be used one its own or combined with other treatments, including steroids. It can also be used as a follow-up treatment for systemic steroids, especially in cases where a patient has developed a reliance on them.
Antihistamines, especially hydroxyzine, can be used if itching is severe. While their effectiveness varies, they may allow healthcare providers to avoid the use of systemic steroid treatment in some cases. Hydroxyzine can also be combined with other antihistamines. Other lesser-known treatments include colchicine, PUVA (psoralen and ultraviolet A) therapy, interferon-alpha, antimalarials, and anti-TNF agents, but these have only been reported as effective in a few individual cases.
In most cases, oral corticosteroids allow for quick healing, and gradually reducing the dose over a month is generally well-tolerated. Continuing low-dose therapy can prevent recurrences. Dapsone may be prescribed initially in less severe cases and can also give good results if steroids aren’t effective. For milder cases, topical corticosteroids may be sufficient. It is also important to treat any other associated diseases if they’re identified, as it could cure Wells syndrome.
What else can Wells Syndrome be?
If you have symptoms of a skin infection, your doctor will need to figure out exactly what’s going on. One common issue is acute bacterial cellulitis, but that usually comes with signs of a general infection like a fever or high white blood cell count. This condition also usually comes and goes. Under the microscope, the tissues affected by bacterial cellulitis often have a lot of swelling and a kind of white blood cell called a neutrophil.
Notably, there are other conditions that can look similar to bacterial cellulitis. For example, some people might have an unusual chronic version of something called erythema migrans. Another similar syndrome is called eosinophilic annular erythema, which many consider to be a part of an umbrella of conditions called Wells syndrome.
