What is Erythema Annulare Centrifugum?
Erythema annulare centrifugum is a skin condition that appears as red, ring-shaped patches. These patches begin as hives-like bumps that grow outwards and clear up in the middle. You might also see a fine skin flake, known as a trailing scale, along the patch’s growing edge. This skin condition is categorized as a ‘reactive erythema’, meaning it responds to various underlying conditions, including cancer. If this skin condition appears due to cancer, it’s known as ‘paraneoplastic erythema annulare centrifugum eruption’. This condition often affects women more and usually appears before a cancer diagnosis and may reappear if the cancer comes back.
The term ‘erythema annulare centrifugum’ was first used in 1916 by Darier, though some authors still use the older term ‘erythema perstans’ to describe similar red, ring-shaped rashes. Despite different names over time, all these terms describe variations of erythema annulare centrifugum. Some skin experts prefer to distinguish between two forms (superficial and deep) as superficial and deep gyrate erythema. Meanwhile, others believe that these two forms are separate conditions and should not share the same name.
Importantly, erythema annulare centrifugum is one of three main types of ‘figurative erythemas’. These are skin conditions that all present as expanding red, ring-shaped patches. However, each type has its own unique clinical presentation and tissue changes under the microscope. If other major types, such as erythema marginatum, erythema migrans, and erythema gyratum repens, are ruled out, erythema annulare centrifugum is a diagnosis of exclusion, meaning it’s only diagnosed once these other conditions have been ruled out.
What Causes Erythema Annulare Centrifugum?
Erythema annulare centrifugum is often a mystery – we don’t really know why it happens. Still, there are many reports linking it to other medical conditions. The good news is that once these underlying conditions are treated, the skin problem also goes away.
What kinds of conditions, you wonder? Well, let’s start with infections:
1. Bacteria: Erythema annulare centrifugum has been associated with bacteria like Escherichia coli. In one case, a person was cured of erythema annulare centrifugum three weeks after their urinary tract infection was treated. It’s also been linked to the bacteria that causes strep throat and tuberculosis.
2. Parasites: Sometimes, a specific parasite can be the cause and once it is cleared with medication, the skin problem also clears.
3. Fungi: Fungal infections like Candida albicans, Blue Cheese Penicillium, and others are also linked with this skin condition.
4. Viruses: It’s been noticed in patients who have had Epstein-Barr virus, Molluscum contagiosum, herpes, HIV and SARS-CoV-2.
Certain medications have been known to cause erythema annulare centrifugum. When these medications are stopped, the skin problem tends to go away. These include medications like amitriptyline, azacitidine, chloroquine, cimetidine, estrogen, etizolam, finasteride and more.
Then there are neoplasms, which are abnormal growths in the body that can be either benign (non-cancerous) or malignant (cancerous). In some cases, a skin issue like erythema annulare centrifugum might bring an undiagnosed cancer to light. If the cancer is successfully treated, the skin issue also tends to go away. It’s been associated with a range of cancers, with leukemia and lymphoma being the most common.
We also can’t overlook some other conditions like:
– Recurring appendicitis (inflammation of the appendix)
– Certain liver diseases
– Thyroid diseases (where the skin condition clears up after the thyroid problem is treated)
– Certain patterns relating to a woman’s menstruation cycle
– Hypereosinophilic syndrome, a rare disorder where there are too many eosinophils (a type of white blood cell) in the body
– Knee osteoarthritis
– Stress and pregnancy.
All these are different factors that could potentially cause or worsen erythema annulare centrifugum. But don’t worry, the condition usually gets better once these factors are addressed.
Risk Factors and Frequency for Erythema Annulare Centrifugum
Erythema annulare centrifugum is a condition that doesn’t have a clearly defined incidence and prevalence, as the literature mostly includes case reports and brief reviews. Though it can show up in any age group, it usually appears most often in adults around the age of 40. Occasionally, it can even start as early as infancy. It affects both men and women equally, and there isn’t any known preference by ethnicity. There’s even a rare inherited version called familial annular erythema.
- The literature on erythema annulare centrifugum mostly includes case reports and quick reviews, leading to a lack of a clear incidence and prevalence rate.
- People of all ages can get it, but it usually starts in mid-adulthood.
- Onset can occur as early as infancy, with documented cases up to the ninth decade of life.
- Both genders can get it, and no preference for any ethnicity is known.
- The average age of onset is around 40.
- A rare inherited form of this condition, familial annular erythema, exists.
Signs and Symptoms of Erythema Annulare Centrifugum
Erythema annulare centrifugum is a skin condition that may present with a rash that is neither itchy nor uncomfortable, although for some people it may feel itchy. This reaction could be triggered by an existing health condition, like tuberculosis or Hodgkin lymphoma. These underlying conditions may cause additional symptoms like night sweating, fevers, and shivering. If you have recently started taking a new medication, be sure to pass that information along to your doctor, as it could be related to this skin condition.
The skin rash typically starts as small, smooth lesions that are round or have multiple connecting circles. These lesions can grow slowly, spreading about 2 to 3 millimeters per day, and after some time, the center of the lesion might become clear. Often, you might find a scale-like texture at the inner edge of the rash. In case the growth isn’t uniform, the rash could develop into incomplete arcs or other forms. Typically these rashes appear on the thighs and legs, but can sometimes appear on the face, trunk, or upper arms. The palms of your hands and soles of your feet are usually unaffected.
Not all rashes will have the scale-like texture. Often, they lack crusts or blisters on their surface. However, some cases have been reported where tiny blood vessels have become visible on the skin’s surface and bruises appeared. In some cases, blisters have developed along the edge of the rash paired with itching. If this rash is related to Hodgkin or non-Hodgkin lymphoma, tuberculosis, or autoimmune processes, you might notice lymph nodes swell.
- Details of the skin condition
- It’s tied to other medical conditions/treatments
- Details of associated symptoms
- Importance of seeking medical advice
- Details about where it usually appears on the body
- In exceptional cases, how the condition might appear differently
- Connection with other symptoms
When it appears, the rash’s edges may become elevated. Even though the rash doesn’t typically cause discomfort or itching, this varies from case to case. After the rash resolves, it does not leave scars; however, you might notice some skin discoloration. These individual skin changes can last anywhere from weeks to months without causing any additional body-wide symptoms. In rare cases, some forms of this condition recur annually and could persistently appear and disappear from days to decades.
Testing for Erythema Annulare Centrifugum
If you’re suspected to have erythema annulare centrifugum, a skin condition that causes ring-shaped patches on the skin, doctors will carry out a detailed health history and thorough physical examination. There are also some specific steps to make sure that the diagnosis is accurate:
First, the doctor will try to rule out any other conditions that could be causing your symptoms. If there’s a chance that you may have a form of cancer, the doctor will perform screenings suited to your age to confirm or rule this out. In many cases, it’s unclear what causes erythema annulare centrifugum.
If there’s a possibility that you might have a fungal infection of the nail (onychomycosis), foot (tinea pedis), or body (tinea corporis), the doctor may send samples of your toenails or skin scrapings to the lab for testing. They do this to see if they can find hyphae, structures that indicate fungal infections like tinea or candidiasis (yeast infection).
If there’s a chance this condition could be a result of Lyme disease or syphilis, your doctor may recommend blood tests. These tests can detect certain antibodies in your blood that indicate whether you’ve been exposed to these infections.
In some cases, erythema annulare centrifugum may be associated with conditions such as Sjögren syndrome or systemic lupus erythematosus. To explore this, a test to detect anti-nuclear antibodies can be carried out. These antibodies are often found in people with these conditions.
If there’s a suspicion of tuberculosis, the doctor may perform an anergy panel and a test for purified protein derivative. These tests help understand your body’s immune response and can suggest tuberculosis.
Examining your complete blood count may be beneficial to identify if an infection is causing the condition. The doctor might also arrange for a stool examination, especially if you have gastrointestinal complaints. Certain parasites detected could suggest their association with erythema annulare centrifugum.
If you’re a woman, the doctor may recommend a test for the hormone beta-human chorionic gonadotropin. This hormone is usually produced during pregnancy.
Chest x-rays might be needed if there are symptoms like lung nodules or enlarged lymph nodes in the chest. These findings can suggest the presence of tuberculosis, cancer (either primary or metastatic), sarcoidosis (inflammation that occurs in various parts of the body), or lymphoma (a type of cancer).
Treatment Options for Erythema Annulare Centrifugum
The treatment for a skin condition called erythema annulare centrifugum usually aims to ease any symptoms linked to it and treat the skin lesions if no related issues are identified. If this condition is triggered by another health problem, treating the underlying issue often results in clearing the skin.
One way to manage this condition is through localized therapy. This involves using creams or lotions directly on the skin to relieve symptoms like itching and inflammatory skin patches. Doctors often recommend applying corticosteroid creams on the leading edge of the skin lesions. If itching is present, creams that reduce this sensation can also be applied. Two types of medications often used for treating this condition include corticosteroids and calcineurin inhibitors, specifically tacrolimus and pimecrolimus, which help to calm the immune system response.
In particularly severe cases of erythema annulare centrifugum, such as severe itching, doctors may recommend systemic therapy, which involves medications that work throughout the body. This may include a short course of systemic corticoid medications, which can provide relief from symptoms, and sedating antihistamines, which help with sleep and reduce itching. However, please note that the symptoms often come back when you stop taking these medications. Sometimes, doctors might recommend using antibiotics or antifungal medications, even if the exact cause is not identified, because many infectious diseases have been linked to this skin condition. Some systemic drugs such as fluconazole, azithromycin, erythromycin, and metronidazole have been used to treat erythema annulare centrifugum.
Specific medications like Etanercept, upadacitinib, doxycycline, and apremilast have been documented to help with severe cases of erythema annulare centrifugum. Apremilast is a medication that can enhance the production of an anti-inflammatory protein, interleukin 10, which helps in bringing down inflammation. Roflumilast, another topical medication usually used to treat cases of erythema annulare centrifugum that haven’t responded to standard treatments, has also shown effectiveness.
What else can Erythema Annulare Centrifugum be?
When a physician is trying to diagnose the skin condition called erythema annulare centrifugum, there are numerous other conditions they need to consider. This is because these conditions show similar symptoms. Some of these conditions are related to autoimmune diseases like Sjögren syndrome, lupus erythematosus, and linear immunoglobulin A bullous dermatosis. Common skin conditions such as discoid eczema, psoriasis, seborrheic dermatitis, and pityriasis rosea might also be considered. In some instances, skin changes could even be an indication of internal diseases, such as stomach, ovarian, or breast cancers.
It’s important to note also that there are types of skin conditions that present with rings or circles of redness, somewhat similar to erythema annulare centrifugum. These include the following:
- Erythema gyratum repens: This is a rash that spreads rapidly and is usually linked to an underlying cancer.
- Necrolytic migratory erythema: This condition causes ring-shaped red lesions that blister, erode, and crust, often as a result of a type of pancreatic tumor known as a glucagonoma.
- Erythema marginatum: This type of rash appears in acute rheumatic fever, with pink or red circular lesions.
- Erythema migrans: This is a growing red patch typically associated with a tick bite.
- Erythema multiforme: This condition causes reddish-pink lesions often located on the backs of the hands and tops of the feet.
- Tinea: Standard fungal infection resulting in expanding, itchy, and scaly lesions.
- Granuloma annulare: This condition presents a circular rash with a raised, smooth red edge and clear center.
- Secondary syphilis: This condition may show non-itchy, scaly, rings and plaques.
- Subacute cutaneous lupus erythematosus: This condition typically presents with central discoloration and tiny widened blood vessels in the skin.
- Erythema papulatum centrifugum: This skin condition is characterized by red, itchy patches that grow into rings or arcs on the trunk and limbs, commonly associated with excessive sweating.
What to expect with Erythema Annulare Centrifugum
The outlook for erythema annulare centrifugum, a skin condition that causes ring-shaped rashes, is generally positive, except in cases where it’s linked with cancer or other serious diseases. On average, this skin condition lasts about 11 months. However, this can vary, lasting from as little as 4 to 6 weeks to as long as 34 years in recurring cases. Most of the time, it goes away on its own without the need for treatment. If erythema annulare centrifugum is associated with a cancerous tumor, the skin rash usually gets better when the cancer is treated. In this case, the outlook depends on the underlying cancer condition.
Possible Complications When Diagnosed with Erythema Annulare Centrifugum
Complications from erythema annulare centrifugum are not usually experienced unless it is linked with another existing health issue.
Preventing Erythema Annulare Centrifugum
Finding and treating the main cause of a skin condition known as erythema annulare centrifugum, including any fungal skin infection, is extremely important. If the condition is linked to pregnancy, it typically gets better after childbirth. If a skin specialist suspects the condition could be due to cancer, they will arrange specific checks suitable for your age to make sure this isn’t the case. It’s unfortunate, but most of the time, the exact cause of this skin condition can’t be determined.
Several treatments have shown benefits in individual case reports. These include applying special creams directly to the skin (topical calcipotriene and tacrolimus), receiving narrowband ultraviolet B light therapy, injecting medicine under the skin (subcutaneous etanercept and interferon-alpha), and taking oral metronidazole medication.
