What is Eosinophilic Pustular Folliculitis?
Eosinophilic pustular folliculitis, or EPF, first identified in 1965 and previously known as Ofuji disease, is a rare skin condition that can affect anyone. EPF can present in three different ways: EPF in infancy (EPFI), classic EPF in adulthood (EPF), and EPF associated with a weakened immune system (IS-EPF).
All three versions of EPF include a specific symptom – itchy skin blemishes caused by pus build-up which are not a result of an infection. These conditions also have a specific finding under a microscope – the presence of a type of white blood cell called eosinophils.
However, despite these similarities, the approaches to treatment and the expected outcomes can vary depending on the type of EPF. This highlights the need for medical professionals to have a thorough understanding of how to diagnose, treat, and manage each subtype of EPF.
This simplified explanation will cover the spread of EPF, the suggested causes, the assumed process of development, the usual symptoms, ways to evaluate the condition, and treatment strategies. It will also emphasize the importance of a team of healthcare professionals in taking care of patients with this skin condition.
What Causes Eosinophilic Pustular Folliculitis?
The exact reason why Eosinophilic Pustular Folliculitis (EPF) occurs is still a mystery, though there are multiple theories that scientists have suggested.
One theory is that certain people may be genetically more likely to get EPF. There are some studies that have found certain signs in the body that may suggest a higher risk of getting EPF. However, more research is needed to fully understand how genes play a role.
Another theory is that EPF is related to the immune system, as it seems to occur more frequently in people living with HIV (Human Immunodeficiency Virus). This theory is also supported by the fact that people who have received organ transplants and are on medicines that suppress their immune system also have a higher chance of developing EPF. Additionally, the presence of a specific type of white blood cells called eosinophils (those cells usually involved in allergy and inflammation situations) in all forms of EPF supports this hypothesis. It’s been suggested that an unusual immune response may cause this inflammation. Some cases have also been related to typical side effects of certain medications.
Treatment for EPF varies a lot and can include medicines that suppress the immune system, antibiotics and light therapy. This suggests that the causes of EPF can vary and might involve multiple factors. It seems like a complicated interaction between the immune system, infectious agents, and a person’s genetic make-up may be at play. Further research is needed to fully understand these complex causes to improve treatments for EPF.
Risk Factors and Frequency for Eosinophilic Pustular Folliculitis
Eosinophilic pustular folliculitis (EPF) is a rare disorder, but people from all over the world can get it, with most cases reported in Japan. The condition tends to affect adults in their 30s and 40s. A type of EPF, known as EPF-I, usually shows up before a child’s first birthday and typically gets better by the time they turn three. Another type, IS-EPF, is often found in people with advanced stages of HIV or AIDS. In fact, about 5% of people with HIV will develop EPF at some point after their diagnosis.
The disorder is more common in males than females, with a ratio of 5 to 1. But getting accurate numbers is difficult because EPF is rare and large-scale studies are lacking.
Signs and Symptoms of Eosinophilic Pustular Folliculitis
Eosinophilic pustular folliculitis (EPF) is a skin condition that appears in three subtypes, all typically showing similar symptoms. People with EPF tend to have cycles of extremely itchy bumps and non-infected pimples located on areas rich in oil glands and hair, like the scalp. These symptoms may also show up following the pattern of a previous skin injury or irritation.
Generally, these flare-ups of EPF last around a week. After treatment, the person may have no symptoms for about four weeks, but then the condition often comes back. There are also uncommon versions of EPF, which may seem like other skin conditions, such as pemphigus foliaceous.
- The first subtype of EPF (EPF-I) usually shows up within the first 14 months of a baby’s life and generally goes away within the first 36 months. This is different from other newborn skin conditions that happen right after birth. EPF-I mostly affects the scalp and seldom shows a ring or wave-like pattern.
- The classic type of EPF mainly appears in middle-aged men, featuring wave-like and ring groups of bumps and pimples on the face, back, and trunk. Oddly, EPF sometimes appears on the hands and feet, or it may show up where another skin condition healed before.
- The third subtype, IS-EPF, mostly shows as scratched and rash-like bumps. Though, it may also arise in the pattern of previous skin irritation or in atypical areas.
Testing for Eosinophilic Pustular Folliculitis
If your skin has bumps filled with pus (a condition known as pustular eruptions), it could be because of a variety of reasons. This might be due to common conditions like acne vulgaris or much rarer ones like graft-versus-host disease.
When you visit your doctor, they will usually ask about your health history, particularly any issues with your immune system that could weaken it chronically. This is important because immune system imbalances often contribute to skin issues like pustular eruptions.
If your red, itchy bumps do not get better with the use of topical steroids (medications applied directly to the skin), your doctor may need to perform further tests. According to Nomura and colleagues, these tests could include looking under a microscope at skin scrapings mixed with potassium hydroxide or mineral oil, performing a complete blood count, testing for HIV, and taking a biopsy including a bump and hair follicle.
Treatment Options for Eosinophilic Pustular Folliculitis
The initial treatment for all three types of Erythroderma (EPF), a skin condition that causes extensive redness and peeling, includes the use of creams containing steroids. Additionally, for a common type of EPF, a medication called indomethacin is often used. This medication has been shown to effectively treat both typical and atypical symptoms of the disease.
In patients with EPF that is linked to a weakened immune system, the main aim of treatment is to restore normal levels of T-cells, which are crucial for immune system function. If the patient’s immune system is weakened due to HIV, a type of treatment called highly active antiretroviral therapy (HAART) can be used. Other medications may also be used to give temporary relief from itching. In cases where symptoms of EPF persist despite treatment, or in cases of severe EPF, other therapeutic options might be considered. These include ultraviolet B (UVB) phototherapy, a type of light therapy, as well as medications such as low-dose isotretinoin, tetracyclines which is an antibiotic, corticosteroids or other anti-inflammatory drugs.
For patients with EPF caused by a drug reaction, the aim of treatment is to alleviate discomforting symptoms such as itching and redness until the condition clears up on its own. Topical steroids (cream or ointment form of steroids) or erythromycin which is an antibiotic are beneficial in this case.
Scabicides, specifically permethrin, a type of medication used to treat parasitic infections, can be useful in treating HIV-related EPF. Permethrin works by interfering with certain processes in the nerve cells.
Antihistamines, which are medications used to treat allergies, can help to reduce itching in cases of HIV-associated EPF. It’s worth noting that sedative antihistamines, which cause drowsiness, can be particularly effective when itching intensifies at night. Medicines like Cyproheptadine and Hydroxyzine can help ease symptoms caused by allergens, while Cetirizine may be useful in treating EPF in infants.
What else can Eosinophilic Pustular Folliculitis be?
According to the diagnostic protocol by Nomura et al., there are several skin conditions that can cause itchy, pustular (pimple-like) skin. These conditions, which a skilled doctor will need to consider, include:
- Bacterial and fungal folliculitis (inflammation of the hair follicles)
- Seborrheic dermatitis (a common skin disease that causes a red, itchy rash)
- Scabies (a skin infestation caused by a mite)
- Other insect bites
- Pustular rosacea (a form of rosacea that causes red, pus-filled bumps)
- Cutaneous T-cell lymphoma (a type of skin cancer)
Other skin conditions, which could also present similar signs or symptoms, are photodermatitis (skin irritation caused by sunlight), papular urticaria (itchy bumps caused by an allergic reaction to insect bites), and Graft-versus-host disease in people who have had an organ transplant.
In addition, a skin condition called EPF-I can look similar to several other conditions in newborns and young children. These include:
- Transient neonatal pustular melanosis (a condition characterized by small blisters and dark spots on the skin at birth)
- Erythema toxicum neonatorum (a harmless condition that results in a rash on a newborn’s face and body)
- Acropustulosis of infancy (recurrent itchy blisters and sores mainly on the hands and feet)
- Kerion (a severe type of fungal infection on the scalp)
- Langerhans cell histiocytosis (a rare disorder that can damage skin, bones and other parts of the body)
- Acne neonatorum (baby acne)
What to expect with Eosinophilic Pustular Folliculitis
The outcome of EPF (Eosinophilic Pneumonia), a lung condition, largely depends on its type. Classic EPF can be a long-term disease with periods of getting better and worse. For EPF-I and IS-EPF, the disease can reliably be expected to resolve with age or by correcting weakened immunity respectively.
Possible Complications When Diagnosed with Eosinophilic Pustular Folliculitis
The complications of all types of pustular skin disease, also known as EPF, are generally mild and uncommon. But, if the skin sores become severe and don’t heal over time, they can lead to scarring and shape changes to nearby tissue. For example, if this happens near the edge of the eyelid, it can lead to a condition called ectropion.
List of complications:
- Scarring
- Changes in the shape of nearby tissue
- Ectropion, a condition where the edge of the eyelid turns outwards
Preventing Eosinophilic Pustular Folliculitis
Current research on a skin condition known as Erythema Pustulosum (EPF) doesn’t provide specific steps to prevent it or clear guidelines on educating patients. Also, no proof has been found yet to show that changing your diet or reducing exposure to certain environments can help manage this skin condition. Even so, doctors recommend that patients with EPF should avoid scratching the affected skin areas too much. Doing so may increase the risk of a bacterial infection in these areas, which are typically filled with a substance called pus that doesn’t contain any bacteria. However, controlling the urge to scratch can be tricky due to the itchy nature of this condition.
