Wells' Syndrome
Wells' syndrome is marked by the sudden appearance of large, red, swollen patches often covered with bumps or blisters. A sensation of itching or burning precedes the eruption.
Wells' syndrome is marked by the sudden appearance of large, red, swollen patches often covered with bumps or blisters. A sensation of itching or burning precedes the eruption.
Wells' syndrome is rare, with fewer than one hundred cases reported worldwide. It mainly affects adults but has also been found in children. Men and women are impacted equally.
Wells syndrome's cause is unknown, but experts believe it is an autoimmune disorder. This is when a person's immune system mistakes the healthy body tissue as an invader then it attacks and destroys it. The disease appears to occur randomly, but it could be genetic in some families.
Itching or burning in one or more areas of the skin is often the first sign, followed by redness and swelling. Pain is common in these skin lesions.
In other patients, multiple new blisters or nodules may appear as the first symptom. The appearance of itchy, ring-shaped patches is another possible sign.
Asthma, joint pain, and fever are possible but uncommon physical manifestations in some patients.
Skin gradually turns from brown-red to blue-gray to greenish-gray over time. The patches normally disappear completely after a few weeks to months; however, they might reappear anytime.
Skin biopsy is the gold standard for diagnosis. It will reveal many eosinophils (white blood cells) in the skin and underlying tissue and inflammation patterns consistent with Wells' syndrome. Similarly, the procedure will rule out other potential causes of inflammation, such as allergies, parasites, dermatitis, insect bites, or Churg-Strauss syndrome.
Treatment
Corticosteroids are the go-to treatment for neutrophilic and eosinophilic dermatoses (skin lesions). In ten percent of cases, corticosteroids cut down on the length and severity of relapses.
Dapsone would cut the length of relapses. However, there is no consensus on how long treatment should last, it's possible that several months would be reasonable if no serious side effects developed. It can be used alone or with other medicines, like corticosteroids. It can also act as a relay for corticosteroids in general, especially in people dependent on corticosteroids.
Antihistamines, especially hydroxyzine for pruritus, have excellent tolerance and may be tried initially because of their effectiveness in 25% of patients.
Recommended general strategy
A gradual decrease in dosage over a month is advisable. Prevention of relapses is possible with continued low-dose corticosteroid medication. Depending on the severity of the inflammation, dapsone may be the first line of defense. It is effective even in the face of resistance to corticosteroids.
IFN-alpha and IFN-beta may be viable alternatives.
Topical corticosteroids may be enough for mild cases. Lastly, it is important to treat an associated disease when diagnosed.
Presently there is no way to prevent Wells syndrome.
References
Toumi A, Yarrarapu SNS, Litaiem N. Wells Syndrome. [Updated 2022 Sep 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK532294/
Cleveland Clinic (2022). Wells Syndrome. Retrieved December 27, 2022, from https://my.clevelandclinic.org/health/diseases/15436-wells-syndrome
