Eosinophilic pustular folliculitis

Alternate names

Ofuji's disease; Ofuji disease; Eosinophilic folliculitis, pustular; EPF; Eosinophilic folliculitis

Definition

Eosinophilic pustular folliculitis (EPF) is a skin disorder characterized by recurring itchy, red or skin-colored bumps and pustules (bumps containing pus).

Cause

The underlying cause of EFP is not known.

Types

There are several variants of EPF including classic eosinophilic pustular folliculitis (mainly occurring in adults in Japan); HIV-associated EPF, also referred to as immunosuppression-associated EPF; and infantile EPF (with onset from birth or within the first year of life).

Signs and symptoms

• This condition characterized by recurring itchy, red or skin-colored bumps and pustules (bumps containing pus).
• The papules mostly appear on the face, scalp, neck and trunk and may persist for weeks or months. EPF affects males more than females.

Diagnosis

• The diagnosis can be supported by the finding of eosinophilia but a skin biopsy is necessary to establish it.
• Skin biopsies reveal lymphocytic and eosinophilic inflammation around the hair follicles.
• The condition is named after the fact that skin biopsies of this disorder find eosinophils (a type of immune cell) around hair follicles.

Treatment

• A variety of treatment options for eosinophilic pustular folliculitis (EPF) have been described with variable results.
• Individualized therapy should be tailored to the type of EPF present.
• The effectiveness of oral nonsteroidal anti-inflammatory drugs (NSAIDs) is well established for many cases of classic EPF (more than 70%), but the reason why remains unknown. There are cases in which NSAIDs are not effective.

For cases of classic EPF, common NSAID options for treatment include indomethacin (orally or topically) and acemetacin. Naproxen has also been used with some success. For classic cases that are not responsive to NSAIDs, other treatment options include:

• dapsone
• topical and systemic steroids
• isotretinoin
• itraconazole
• permethrin
• interferon
• antibiotics

Additional treatment options that may improve symptoms include:

• narrowband UVB phototherapy
• ultraviolet therapy with ultraviolet B or with ultraviolet A
• Psoralen plus ultraviolet A (PUVA)
• calcineurin inhibitors such as tacrolimus ointment
• colchicine
• transdermal nicotine patches

The infantile form tends to respond well to topical corticosteroid therapy.

• It is important to recognize that this form typically resolves on it's own before age 3 in over 80% of cases, so observation or avoiding aggressive treatment is often recommended.

• In people with HIV-associated EPF, antiretroviral therapy tends to greatly diminish symptoms or even eliminate the condition.

Prognosis

• The long-term outlook (prognosis) for people with eosinophilic pustular folliculitis (EPF) depends on the type of EPF present (classic, infantile, or immunosuppression-associated).
• Classic EPF, which occurs mainly in adults, is generally chronic and recurrent but benign.
• Individual lesions typically last more than 1-2 weeks, and relapse every 3-4 weeks.
• Except for skin involvement, people with classic EPF are generally in good health with no systemic symptoms.
• Infantile EPF generally also runs a benign course, and resolves on its own by 3 years of age in over 80% of cases.
• It is important to recognize that this form is self-limiting so as to avoid unnecessary aggressive treatment in affected infants.
• Children with this form also generally remain in good health without associated problems, although a possible association with atopy (tendency to develop allergies) has been speculated.
• The immunosuppression-associated type of EPF is mainly associated with HIV infection, hematological malignancy (blood cancers), or other cancers, so recognizing that a person has this form of EPF is important.
• The overall prognosis beyond the skin symptoms largely depends on the prognosis associated with the underlying condition.

NIH genetic and rare disease info

• NIH info on Eosinophilic pustular folliculitis

Eosinophilic pustular folliculitis is a rare disease.

Eosinophilic pustular folliculitis Resources
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