What is Necrolytic Acral Erythema?
Necrolytic acral erythema is a rare skin condition largely linked to chronic infection with hepatitis C virus (HCV). It was first identified in 1996 and is marked by painful, red, eroding skin patches, which can sometimes turn into sores filled with fluid. These patches mainly occur on the extremities like hands and feet. The exact cause of this condition is unclear, but it might have something to do with the interaction between lack of certain nutrients, especially zinc, and chronic liver disease.
People often see signs of necrolytic acral erythema when they’ve been infected with HCV for a long time with no treatment or not managed properly. The skin changes are typically clearly outlined, red, eroding patches with a superficial skin cell death and a crusty surface. The course of this condition is usually long-term with periods of increasing and decreasing symptoms.
Diagnosis involves a thorough check-up to find out the underlying causes and any metabolic disturbances. Testing for HCV is particularly emphasized. Sometimes, analysis of a small skin sample may be needed to tell necrolytic acral erythema apart from other similar skin conditions. If not managed correctly, the condition can continue and lead to significant discomfort and secondary infections due to the damage to the skin’s protective layer. Treatment focuses on addressing the HCV infection and the skin changes. Taking zinc through the mouth has proven to be significantly effective.
Even though it seems to be closely linked to HCV, necrolytic acral erythema is still not well understood and not often reported. This makes it hard to figure out how common it is and how often it occurs.
What Causes Necrolytic Acral Erythema?
Necrolytic acral erythema is a skin condition that is mainly linked with the Hepatitis C virus (HCV) infection. The specific way that HCV infection causes necrolytic acral erythema is not fully understood, but it’s believed to involve a few key elements.
The virus itself may cause changes in the skin directly, or it might set off reactions from the body’s immune system leading to these changes. Additionally, metabolic disorders linked to liver problems might be a part of the process. All in all, the Hepatitis C virus plays a major role in this skin condition.
Risk Factors and Frequency for Necrolytic Acral Erythema
Necrolytic acral erythema, a skin condition, was initially observed in regions like Egypt with high rates of untreated Hepatitis C (HCV) infection. After its discovery, this condition has been identified in various parts of the world including the Middle East, Asia, Europe, and North America. Because this disease is rare and often underreported, we don’t know exactly how common it is.
The link between this skin condition and chronic HCV infection has led to its increased recognition in regions where there are high Hepatitis C rates. This condition typically affects adults between the ages of 40 and 60, and is not more common in one gender over the other.
Chronic HCV infection is largely associated with necrolytic acral erythema. However, having this skin condition doesn’t necessarily mean that the patient will have higher viral load.
Signs and Symptoms of Necrolytic Acral Erythema
Necrolytic acral erythema is a condition that can be linked to Hepatitis C (HCV), but often, people with this skin problem don’t know they have HCV. The condition presents differently in different people because it varies in stages and duration. It is marked by painful, red, eroded patches, and sometimes blisters, mainly on the hands and feet.
When examined, three stages can be observed. Here’s a simplified description of what happens:
- In the initial stage, the issue starts as small, red spots or patches that slowly grow and develop a crusty center due to cell death.
- In the well-developed stage, these spots combine to form distinct, thick, dark patches with attached scales.
- As the condition moves into the late stage, the patches become more defined, thinner, and darker in color.
Testing for Necrolytic Acral Erythema
When looking into necrolytic acral erythema, a condition that causes lesions on the skin, it’s essential to perform thorough tests to find any hidden underlying issues or metabolic disruptions. Spotting necrolytic acral erythema early on is critical for diagnosing and treating any conditions that may have led to it, specifically the Hepatitis C virus (HCV) infection. This is important because in over 75% of cases where necrolytic acral erythema is found, the Hepatitis C virus is also detected for the first time.
Although there’s no specific lab test to definitively diagnose necrolytic acral erythema, a series of biochemical tests are the next best thing. These may include measuring serum zinc levels (the liquid part of your blood). These levels are typically normal, but in some cases have been reported to be low. Professionals may also conduct tests assessing amino acid and albumin (a protein made by your liver) levels, which sometimes show as abnormal in patients. Normal levels of glucagon (a hormone made by the pancreas that increases your blood sugar levels) can make it easier to distinguish between necrolytic acral erythema and similar conditions like necrolytic migratory erythema.
Tests checking liver function, including transaminase (enzymes located in liver cells) levels and total serum bilirubin (a compound found in the blood that helps to check bile production), are particularly crucial, especially for patients with Hepatitis C. In fact, unusual results for liver function tests and ultrasound studies are relatively common, reported in 84.3% and 76.9% of cases, respectively. Evaluations may also be undertaken to rule out other conditions that present themselves in a similar way to necrolytic acral erythema. This thorough, multi-step examination process helps provide the most accurate detection and suitable treatment plan for the patient.
Treatment Options for Necrolytic Acral Erythema
Necrolytic acral erythema is a skin condition that’s usually managed by addressing both the root cause and the skin symptoms. For instance, in patients who have a Hepatitis C Virus (HCV) infection, certain antiviral medicines like ribavirin and interferon-alpha have been found to improve the condition, even if the virus remains present in high quantities in the body. Using interferon-alpha alone has also had positive effects.
One form of treatment known to be quite effective against necrolytic acral erythema is oral zinc therapy. This is even applicable to patients who don’t have a zinc deficiency. Fascinatingly, it has been found that zinc therapy can also boost the effectiveness of interferon treatment in HCV patients who have this skin condition.
Other treatments have also been explored, with different levels of success. Medications like topical and systemic corticosteroids, and another medicine called topical tacrolimus, have been tried with mixed results. There have also been trials with phototherapy methods, which mean using light to treat a condition. These methods, including a treatment called psoralen plus ultraviolet A therapy, and another called narrowband ultraviolet B therapy, have been tested but they have not shown any benefit so far.
What else can Necrolytic Acral Erythema be?
Necrolytic acral erythema, a skin condition, could be mistaken for many other skin problems that show similar signs such as redness, flaking skin, and patches that resemble psoriasis. It’s very important to accurately identify the issue to ensure correct treatment. Some other conditions that might look like necrolytic acral erythema include:
- Acrodermatitis enteropathica
- Niacin deficiency dermatitis
- Biotin and fatty acid deficiencies
These health issues usually appear on particular skin areas and don’t come with body-wide symptoms, which helps doctors tell them apart from necrolytic acral erythema.
Psoriasis often appears as red patches with silvery scales, often seen on parts of the body like elbows, knees, scalp, and the lower back, while necrolytic acral erythema would show dark, wart-like scales and doesn’t have clear areas in the middle of the patches. Sometimes tests from the affected skin are needed to positively identify it and rule out psoriasis.
Another skin condition, lichen simplex chronicus, results in thick, flaking patches due to consistent scratching or rubbing. The patches in this case are very clear, look like lichen, and cause severe itching. Unlike necrolytic acral erythema, it doesn’t show signs of tissue death and isn’t linked to Hepatitis C Virus (HCV) but late-stage necrolytic acral erythema might appear similar. The critical difference is found in their history of chronic itching and a physical examination but a biopsy could be required for identification where necessary.
Erythrokeratoderma appears as hard, red patches often in a map-like or straight shape, typically seen in infants or young children. Unlike necrolytic acral erythema, it shows symmetric distribution and is usually inherited.
Nummular eczema looks like red, round, flaky patches, which often come with itching and discomfort. The lack of very clear patches and wart-like scales seen in necrolytic acral erythema help diagnose nummular eczema.
Additionally, a condition called dermatophytosis appears as red patches with central clearing and flaking at the edges, typically caused by fungal infections. The lack of central clearing along with unique features seen under the microscope in necrolytic acral erythema aids in ensuring a correct diagnosis.
What to expect with Necrolytic Acral Erythema
The outcome of necrolytic acral erythema, a skin disease, can vary greatly. Many factors influence this outcome, including the identification of its cause, how effective the treatment is, and how quickly the disease is diagnosed. As this skin condition often goes hand-in-hand with Hepatitis C Virus (HCV) infection, successful treatment of the HCV infection can greatly improve the condition of the skin and overall health.
However, there have been some cases where the skin lesions caused by necrolytic acral erythema came back after stopping a specific treatment for HCV known as interferon. It’s also been noticed that the return of these skin problems might coincide with the return of liver disease symptoms and the resurgence of the HCV virus.
There are conflicting reports on whether there’s a relationship between liver disease and the severity of the skin condition. A delay in diagnosing necrolytic acral erythema can make the outcome worse due to its rarity and the broad range of potential diagnoses.
Possible Complications When Diagnosed with Necrolytic Acral Erythema
The main challenges related to necrolytic acral erythema come from its long-lasting and resurfacing skin lesions which have a tenancy to be chronic, predominantly if the Hepatitis C infection that usually causes the condition isn’t properly kept in check. These lesions can cause a lot of discomfort and negatively affect the person’s standard of living. More notably, these lesions have an inclination to get secondary infections, especially if the skin’s protective layer is broken due to ulcers or scratching.
Repeated or long-lasting necrolytic acral erythema lesions frequently lead to scarring and postinflammatory pigmentation. This can be problematic for patients from a cosmetic standpoint because necrolytic acral erythema generally impacts the hands and feet. If the case is severe, it can impact the individual’s ability to move and walk, thus, impacting their quality of life further.
Necrolytic acral erythema has a strong connection to chronic Hepatitis C, a condition that can cause serious complications such as cirrhosis, liver failure, and a form of liver cancer called hepatocellular carcinoma, if not properly managed. This relationship highlights the significance of managing the primary Hepatitis C infection when treating necrolytic acral erythema. Problems arise when Hepatitis C treatment is stopped or proves ineffective, leading to the reappearance of necrolytic acral erythema, making the overall illness management more complicated.
Complications:
- Persistent and resurfacing skin lesions
- Pain and discomfort
- Secondary infections in the skin
- Scarring and postinflammatory pigmentation
- Impaired mobility
- Impact on the patient’s standard of living
- Complications from untreated Hepatitis C including cirrhosis, liver failure, and liver cancer
- Relapse of necrolytic acral erythema
Preventing Necrolytic Acral Erythema
One of the best ways to prevent a skin condition called necrolytic acral erythema is to regularly check for and treat Hepatitis C virus (HCV), as it can lower the risk of this skin issue developing. It’s important for patients to understand the connection between HCV and necrolytic acral erythema, as well as how crucial it is to keep their liver healthy and follow the treatment plans suggested by the doctor. The first indicator of this skin condition could be reddish and flaky skin abnormalities on hands and feet, so identifying and reporting these symptoms early can lead to quick medical attention and care.
Educating oneself about good skincare practices and never missing follow-up visits to the doctor can help manage the condition and prevent it from getting worse. Understanding and participating actively in your health care not only can lead to better outcomes but can enhance your quality of life. Remember, empowered patients are often the ones who see the most improvements in their health.
