What is Sneddon-Wilkinson disease?

Sneddon-Wilkinson disease is also known as subcorneal pustular dermatosis. It is a rare, chronic, relapsing pustular eruption characterized by subcorneal pustules with neutrophils on histology. It was initially described by Sneddon and Wilkinson in the 1950s. It is more common in middle-aged and older women, but it has also been observed to affect children.

Causes of Sneddon-Wilkinson disease:

The cause of Sneddon-Wilkinson disease is unknown. However, it is linked to a few other conditions. The most common are:

IgA monoclonal gammopathy
Multiple myeloma
pyoderma gangrenosum

Other less common related disorders include rheumatoid arthritis, lupus erythematosus, hyperthyroidism and hypothyroidism, polycythemia rubra vera, and SAPHO syndrome.

Clinical features of Sneddon-Wilkinson disease:

Sneddon-Wilkinson disease is distinguished by the presence of many soft pustules on the skin surface. The pustules are typically a few millimeters in diameter and are frequently organized in annular, circinate, or serpiginous forms. They typically develop on the trunk, especially in skin folds like the armpits and groin. They can appear on apparently normal skin; however, they are typically found within a red patch. The pustules disappear after a few days and are replaced with fine scale until another relapse occurs and new pustules develop. It may flare up for a few weeks, then disappear for months or years before reappearing.

Diagnosis of Sneddon-Wilkinson disease:

Other dermatological disorders may resemble Sneddon-Wilkinson disease. The patient’s history, physical examination, skin biopsy, and cultures may typically be used to distinguish Sneddon-Wilkinson disease from other disorders. A skin biopsy is commonly used to confirm the diagnosis.
Blood tests usually involve a general screening, such as a blood count, calcium levels, and liver function tests, as well as protein electrophoresis.

Treatment of Sneddon-Wilkinson disease:

The goal of treatment is to prevent complications. Dapsone is often effective, with lesions disappearing after a month. Ongoing maintenance at a reduced dose is sometimes necessary.

Other treatment options include:

Acitretin
Sulfapyridine or sulfamethoxypyridazine
Phototherapy
Colchicine
Ciclosporin or other immune suppressants, such as mycophenolate mofetil
Biological response mediators, including infliximab and adalimumab

Systemic steroids typically do not work effectively and may even trigger a flare-up of subcorneal pustular dermatosis.

Written by:

Atheer Alhuthaili, Medical Student.

Revised by:

Naif Alshehri, Medical Intern.

References:

DermNet.

UpToDate.

Medscape.