Ofuji disease


Ofuji disease

Description, Causes and Risk Factors:

Alternative Name: Eosinophilic pustular folliculitis.

Abbreviation: EPF.

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.

  1. HIV-associated EPF.

  2. Infantile EPF.

Classic eosinophilic pustular folliculitis also known as Ofuji's disease is a rare inflammatory disease that presents mainly on seborrheic areas with erythematous papulopustular lesions in annular configurations. It mostly affects male Asian patients in the third and fourth decades. Most cases are not associated with systemic disorders apart from peripheral blood eosinophilia. Histology shows an infiltrate of eosinophils and lymphocytes.

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.

The causes of all three types of EPF remain unknown. Some researchers suggest that the condition is caused by an allergic reaction to dermatophytes (parasitic fungi that cause skin infections) or saprophytic fungi (fungi that live on decaying or decomposing organic matter), such as Pityrosporum ovale. Also, bacteria similar to Leptotrichia buccalis were found in one biopsy specimen of a patient with HIV-associated EPF.

The main features of the EPF are the reported development of crops of pruritic follicular papulopustules with a red hallow in fairly well defined areas. The skin lesions have tendency to central involution and peripheral extension. Fresh lesions appear at the periphery as old one faded in the center. Beside plaque formation scattered papulopustules are also present.

Symptoms:

The symptoms for all three variants of the disease are generally the same. About 20% of patients suffer from reddish bumps and/or pimples on the hands or feet, which may be the first signs of the disease.

Patients experience dandruff or dry scaly skin on the head and trunk of the body. Papulopustules (white-headed pimples that are filled with pus) usually appear around hair follicles on the face and trunk, although the extremities (arms and legs) may also be involved. The classic form tends to affect the palms and soles of the feet. In children, the scalp is more frequently involved. Individual papulopustules may be larger in the classic form (up to 20 to 50mm in diameter) than the HIV-associated or infantile form, which are typically about one to three millimeters in diameter.

The infection may itch or be tender. However, the classic variant of the disease is typically less itchy than the other two forms.

Uncommon symptoms, including nonfolilcular papules (small lesions on the skin that are not near hair follicles) and hives are often seen in infants and HIV patients.

Diagnosis:

The following disease should be taken into consideration for diagnosis: Pyoderma, dermatophytosis, impetigo herpetiformis, psoriasis Pustulosa, acrodermatitis continua, dermatitis herpetiformis, eczematous dermatitis, candidiasis, subcorneal pustular dermatosis.

The diagnosis is made when typical skin lesions have a compatible histology. As this is a folliculocentric process, serial sections may be needed to find the affected follicle. Peripheral blood eosinophilia supports the diagnosis.

An etiological treatment is unavailable justifying the wide range of therapies with variable success. For localized disease topical corticosteroids seem adequate. With generalized disease, most of the literature favors indomethacin. This drug is generally well tolerated and the response takes 1 to 2 weeks.

Treatment:

Because the origin and the development of eosinophilic pustular folliculitis (EPF) are not fully understood, there is currently no established treatment regimen, and no scientific studies have been performed to determine the most effective treatment for this condition. Treatment generally focuses on decreasing the inflammation and itching.

Corticosteroids both systemic and topical application is effective. Dapsone is reported effective in some cases.

HIV patients 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. Additional treatment options may include oral itraconazole along with an oral sedating antihistamine before sleep if pruritus (itching) is severe, oral metronidazole, and ultraviolet therapy.

Ultraviolet therapy has also been used to treat EPF. Treatment with ultraviolet B or with ultraviolet A and a photosensitizing chemical called psoralen may be beneficial in HIV patients. However, skin lesions typically recur once the phototherapy id discontinued.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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