What is Neutrophilic Eccrine Hidradenitis?

Neutrophilic eccrine hidradenitis (NEH) is a rare, non-dangerous condition which was first identified due to skin rashes in patients with a type of blood cancer known as acute myelogenous leukemia (AML), undergoing systemic chemotherapy. This condition is typically seen as a response to certain events happening in the body and is often associated with other diseases like blood cancers, Hodgkin lymphoma, and solid tumors.

Since it was first recognized in 1982, NEH has often been described alongside other skin conditions categorized under ‘neutrophilic dermatoses.’ Neutrophilic dermatoses refer to a group of skin disorders in which neutrophils, a type of white blood cell, are found in the skin’s primary layer, without any signs of infection being present.

What Causes Neutrophilic Eccrine Hidradenitis?

Neutrophilic eccrine hidradenitis is a harmless skin condition that goes away on its own, but the exact cause isn’t known. With this condition, cells called neutrophils gather around sweat glands. It has been associated with certain medications, including acetaminophen, minocycline, granulocyte colony-stimulating factors, cyclophosphamide, methotrexate, carbamazepine, cetuximab, BRAF inhibitors, bleomycin, methotrexate, 5-fluorouracil, and antiretroviral medications.

When doctors suspect neutrophilic eccrine hidradenitis, they usually first check for infectious diseases. Nevertheless, even in some cases of bacterial and viral infections, the lab results from skin sample tests were similar to those of neutrophilic eccrine hidradenitis. Also, it’s noteworthy that damage to sweat glands due to heat can result in this condition, particularly in children.

Risk Factors and Frequency for Neutrophilic Eccrine Hidradenitis

The exact rate of neutrophilic eccrine hidradenitis, a medical condition, is not well researched or known. However, it has been observed to occur slightly more often in males. It’s been reported in a wide age range, from infants as young as six months old to elders up to 79 years of age.

Signs and Symptoms of Neutrophilic Eccrine Hidradenitis

Neutrophilic eccrine hidradenitis is a skin condition often seen in patients with acute myelogenous leukemia who are undergoing chemotherapy, especially those using the drug cytarabine. This condition can appear as soon as two days or as long as two years after starting chemotherapy. However, it’s also been seen in those with the same type of leukemia who have not undergone chemotherapy.

People with this condition often show red bumps and patches (known as erythematous papules and plaques) most commonly on the face, back, body, and limbs. Regardless of the patient’s past medical history, they often have a fever alongside either one or multiple skin lesions described as dark red or purple spots, bumps, or patches.

This condition usually shows up on the body and limbs. Some people may not show any symptoms, while others may have pain and tenderness in the areas of the skin lesions. Given its varying presentations, it can be difficult to distinguish neutrophilic eccrine hidradenitis from other skin conditions (neutrophilic dermatoses) that are commonly associated with cancer.

This condition could be confused with other skin conditions including:

Acute febrile neutrophilic dermatosis (Sweet syndrome)
Acute urticaria
Graft versus host disease
Erythema nodosum
Erythema multiforme
Cellulitis
Erysipelas

Testing for Neutrophilic Eccrine Hidradenitis

To accurately diagnose neutrophilic eccrine hidradenitis, a specific type of skin condition, your doctor will likely take a sample of your skin using a method known as a skin punch biopsy. This procedure lets them look at the skin sample under a microscope, where they’d expect to find a large amount of neutrophils (a type of white blood cell) in the sweat gland area, along with tissue damage and swelling in the skin.

Your doctor might also order a complete blood count, or CBC. This is a standard blood test that counts the different types of cells in your blood. This helps confirm the diagnosis and also helps to check for any possible underlying blood disorders that might be linked to the skin condition.

Other tests might include checking for inflammation in the body by measuring the levels of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). These are substances that can build up in the bloodstream when there’s inflammation in the body.

Finally, a tissue culture test might be done. This involves taking a small sample of tissue and allowing any microorganisms (like bacteria or viruses) present to grow in a lab. This can help identify if any infections are contributing to your symptoms.

Treatment Options for Neutrophilic Eccrine Hidradenitis

Neutrophilic eccrine hidradenitis is a rare skin disorder that doesn’t have a universally accepted treatment. Generally, the condition resolves itself and doesn’t require any specific therapy. Most of the time, the skin rashes or lesions disappear on their own within about a month.

There’s some debate about whether steroids, either applied to the skin (topical) or taken by mouth (systemic), are effective. It is important to note that steroids should be used carefully if the patient has a low number of a type of white blood cells called neutrophils, which help the body fight off infections. Treating any accompanying symptoms such as fever and pain is highly recommended.

Colchicine, a medication often used to treat gout, has been shown to be effective in treating neutrophilic eccrine hidradenitis in patients who are otherwise healthy. However, this is still being researched.

A common occurrence with neutrophilic eccrine hidradenitis is that it can come back in patients undergoing chemotherapy. In such cases, if it is found that a specific chemotherapy drug triggers the condition, one successful method of managing it has been to use Dapsone, an antibiotic commonly used to treat skin conditions. This is given daily for two days before the patient is treated with the chemotherapy drug again. However, this is based on individual case reports, and further studies are needed to confirm its effectiveness.

When a patient presents with certain skin conditions or issues, doctors may consider several potential diagnoses. These include:

Acute or chronic urticaria (also known as hives)
Vasculitis syndrome (an inflammatory disorder of the blood vessels)
Drug eruptions (skin reactions due to medications)
Erythema multiforme/nodosum (specific inflammatory skin conditions)
Leukemia cutis (skin lesions caused by a type of cancer)

What to expect with Neutrophilic Eccrine Hidradenitis

It’s common for symptoms to come back in patients who are undergoing chemotherapy. However, a condition called neutrophilic eccrine hidradenitis, which affects the sweat glands, is usually temporary and doesn’t make the outlook for the specific cancer any worse.