What is Acrokeratosis Paraneoplastica?
Bazex syndrome, also known as acrokeratosis paraneoplastica or acrokeratosis neoplastica (AN) syndrome, is a rare condition. It is associated with an unusual skin condition similar to psoriasis that affects the extremities (hands and feet), and is often connected with internal cancers, specifically squamous cell carcinoma of the upper parts of the digestive and respiratory systems. However, it’s important to note that nearly any cancer involving squamous cells – the cells that form the outermost layer of our skin – could be linked to this syndrome.
Therefore, patients with this syndrome need comprehensive medical testing to determine where the cancer is. The skin lesions, or abnormal areas, are usually noticed before the underlying cancer is diagnosed and often go away on their own after the cancer is fully treated. If the primary cancer is not treated, not only is the patient’s outlook poor, but the skin lesions won’t get better either.
What Causes Acrokeratosis Paraneoplastica?
The exact cause of Bazex syndrome is still unclear. However, research by Bolognia reported that in 67% of the cases, skin lesions were an early sign of an underlying cancer diagnosis. On the other hand, in 15% of the cases, these skin symptoms appeared after the cancer was diagnosed. There are certain risk factors associated with Bazex syndrome, including tobacco and alcohol use.
Risk Factors and Frequency for Acrokeratosis Paraneoplastica
Appendicitis most frequently affects middle-aged white males in their 40s to 70s. It’s quite rare for females to experience this condition.
Signs and Symptoms of Acrokeratosis Paraneoplastica
The typical symptoms of this condition include skin eruptions that are red and tender but not often itchy. These eruptions often appear on extremities such as fingers, toes, ears, and nose. In many cases, changes to the nails, similar to those seen in individuals with psoriasis, are the first sign of the condition.
Eruptions can also spread to other parts of the body as the underlying disease progresses, encompassing the cheeks, elbows, knees, and trunk. The most common sites for related malignancies or cancers are the neck and head area.
- Squamous cell cancers of the throat, esophagus, and voice box are often associated (in about 39% of the cases).
- Squamous cell lung cancer (11%)
- Lung adenocarcinoma (4%)
- Gastrointestinal adenocarcinoma (8%)
- Genitourinary tumors (5%)
- Lymphomas (type of cancer)
In most cases (around 63%), skin lesions appear approximately a year before the tumor is diagnosed.
As Bazex et al. found, the disease typically develops in three stages. In the undetectable first stage, symptoms are primarily seen in areas like earlobes, the helix of the ear, the tip of the nose, fingertips, or nails. In this stage, it’s common to see blisters, changes underneath the nail, or dystrophic (abnormal growth) nails. These nail changes can include ridging, yellow discoloration, nail separation, growth of skin underneath the nail, and, in more severe cases, shrinking of the nail plate.
The later stages involve eruptions spreading to the palms, soles, cheeks, forehead, elbows, and knees. In the likely final stage, the redness of the skin can spread to the trunk if the underlying tumor progresses.
During a physical exam, doctors have to be vigilant of signs that may signify the presence of a cancer. These indicators could include:
- Itching
- Dry, scaly skin (Ichthyosis)
- Nodules (or lumps) in the belly button area (Sister Mary Joseph nodule)
- Enlarged fingertips (Clubbing)
- An increased number of skin growths (Sign of Leser-Trelat)
- Rectal shelf (Blummer shelf)
- Hard, enlarged lymph node above the collar bone (Virchow’s nodes)
Testing for Acrokeratosis Paraneoplastica
If your doctor suspects that you might have Bazex syndrome, they will initially ask about your health history and do a physical exam. They may also order several tests to get a clearer picture of what’s going on in your body. These tests can include a closer look at your ears, nose, and throat, a chest x-ray, and a blood test that will give information about the count of different blood cells. Apart from that, to check the amount and type of proteins in your blood and urine, protein tests might be required. Other tests can include a measure of how quickly your red blood cells settle at the bottom of a tube (erythrocyte sedimentation rate), a review of the substances in your blood that can indicate your overall health (biochemistry profile), and checks of certain proteins that may indicate cancer (tumor markers). A stool test can also be done to check for hidden blood.
For a detailed view of your internal organs, your doctor might recommend imaging tests. These can include a special type of x-ray that uses barium to highlight your gastrointestinal tract, a regular chest x-ray, an ultrasound of your abdomen and pelvis, and a computed tomography (CT) scan of your chest, head/neck, and abdomen. Magnetic resonance imaging (MRI) may also be used to get detailed images of your chest, head/neck, and pelvis. A mammography, which is a specialized breast imaging that uses low-dose x-rays to detect cancer, might also be suggested. A positron emission tomography (PET) scan could also be used; this imaging test helps reveal how your tissues and organs are functioning.
Depending on your symptoms, additional tests may be necessary. These might include endoscopy to examine your digestive tract, cystoscopy to view the inside of your bladder, bronchoscopy to explore your lungs, or even a biopsy, where a small sample of your bone marrow or lymph nodes are removed and analyzed under a microscope.
Treatment Options for Acrokeratosis Paraneoplastica
The best way to get rid of the skin lesions seen in Bazex syndrome, a paraneoplastic skin condition, is to effectively treat the underlying cancer. These types of skin lesions don’t usually improve with standard treatments for inflammatory skin diseases. However, some treatments including Vitamin D3, salicylic acid, and topical or systemic steroids have shown improvement.
Topical treatments that have been reported include corticosteroids like clobetasol 0.05% or betamethasone 0.01%, salicylic acid 10% mixed with vaseline, itraconazole, isosorbide dinitrate, fluconazole, cephalexin, keratolytic, neomycin, nystatin, zinc ointment, antibiotics, and emollients. Some suggest using oral dexamethasone at 10 mg/day which has shown to benefit the skin lesions.
In the past ten years, there have been a few reports suggesting that retinoids, both in systemic and topical forms, may help to relieve these skin lesions. Acitretin, a particular type of retinoid, has reportedly been effective in improving acrokeratosis paraneoplastica in a patient with an untreatable primary cancer. These Vitamin A derivatives can also be combined with oral corticosteroids. In cases where the skin lesions are localized, there are suggestions that a treatment combination of psoralen and ultraviolet light therapy may be helpful.
Lastly, there have been some reports indicating that taking supplemental zinc could help improve these skin lesions.
What else can Acrokeratosis Paraneoplastica be?
When diagnosing certain skin conditions, doctors usually compare the symptoms with those of diseases like psoriasis and other similar types of dermatitis. They also have to consider other conditions like Pityriasis rubra pilaris, Reiter’s syndrome, and tinea manuum when examining the symptoms of skin lesions that occur on the hands and feet. According to some reports, patients with systemic lupus erythematosus, an autoimmune disease, may also show skin symptoms similar to a condition called acrokeratosis paraneoplastica.
What to expect with Acrokeratosis Paraneoplastica
The effectiveness of treating acrokeratosis paraneoplastica, a skin condition, largely depends on the primary cancer’s response to treatment. If the cancer does not respond or is in an advanced stage, the skin symptoms usually do not improve.
For patients diagnosed with cancer that has already spread to the lymph nodes in the neck, the outlook is usually not positive. But it’s important to note that the serious health effects of this condition are primarily due to the underlying cancer and not the skin condition itself.
Although treatments exist, their results vary greatly from person to person, are often unpredictable, and generally, do not bring satisfactory improvement.
Possible Complications When Diagnosed with Acrokeratosis Paraneoplastica
Aside from the skin abnormalities, acrokeratosis paraneoplastica, a skin condition, doesn’t typically cause any complications on its own.
Preventing Acrokeratosis Paraneoplastica
Patients should be informed about the fact that their disease is paraneoplastic, meaning it’s associated with cancer. They should also be told that usually, the skin problems they are experiencing will clear up after the primary cancer has been fully and effectively treated.
