Eosinophilic pustular folliculitis (EPF) is a recurrent skin disorder that causes bumps and/or pimples to form near hair follicles. Clumps of white blood cells called eosinophils form these skin abnormalities. While the condition is not life threatening, it may cause intense itching.
There are three types of EPF: the classic EPF (Ofuji disease), HIV-associated EPF, and infantile EPF. All three types cause papulopustules or white-headed pimples that are filled with pus to form around hair follicles near the head and trunk. Patients also suffer from dry, scaly skin on the head and trunk that may be itchy or tender. Because little is known about the disease, it is unclear whether these three conditions are interrelated forms of a single disease, or if they are three different diseases.
Classic EPF mainly occurs in Japanese people living in Japan. While the exact incidence of classic EPF remains unknown, it is considered an uncommon disorder. Most individuals who have the classic form of the disease develop the condition when they are 20 to 40 years old. In classic cases, common treatment options include nonsteroidal anti-inflammatory drugs (NSAIDS) like indomethacin (orally or topically) or acemetacin, as well as topical and systemic steroids, isotretinoin (retinoid), itraconazole (antifungal), permethrin (insecticide), and antibiotics like dapsone. Unfortunately, classic EPF usually continues for years with recurrent relapses and remissions. Some patients are able to achieve long-lasting remissions with indomethacin alone or in combination with dapsone.
HIV-associated EPF occurs in patients with advanced HIV. This type of EPF may develop at any age and it is considered the most common form. Nearly 10% of HIV patients develop EPF, according to one survey. HIV patients who develop EPF should begin highly active antiretroviral therapy (HAART) if they are not already receiving treatment. HAART will help reconstitute (restore) the immune system of HIV patients. Other treatments are available, but symptoms usually recur once treatment is discontinued.
Infantile EPF occurs in infants and children. Most individuals who have the infantile form of the disease develop the condition within the first year of life. However, some babies are born with the condition. The infantile type of EPF responds well to topical corticosteroids like hydrocortisone.
Eosinophilic pustulosis of the scalp in infancy/childhood is a recurrent skin condition that can be relieved by topical corticosteroids.
According to the National Institutes of Health (NIH), EPF is a rare disease. The most common variant is HIV-associated EPV. However, the exact incidence of the disease is unknown.
Men are five times more likely to develop EPF than women. However, since the cause remains unknown, more research is necessary to determine the reason behind this.
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