What is Alopecia Totalis?
Alopecia areata, often referred to as AA, is a long-term disease where your immune system mistakenly attacks your hair follicles, causing hair loss. This reaction is primarily carried out by a type of white blood cell known as T-lymphocytes. The disease usually results in hair loss in small, round patches on the scalp, a condition known as alopecia areata focalis. A more extreme subtype of this disease is called alopecia totalis, which leads to total hair loss on the scalp.
In more severe cases, a person may lose all the hair on their body, a condition known as alopecia universalis. People with alopecia totalis have a less favorable chance of their hair regrowing, and are more likely to not respond to treatment.
What Causes Alopecia Totalis?
Both genes and environment play a role in the development of Alopecia Areata (AT), a condition where hair falls out in small patches. Studies on twins and families show that genes likely play a part in getting the disease. For instance, research on identical twins shows that if one twin has the disease, the other one also has it 55% of the time. Furthermore, about 20% of people with Alopecia Areata have a family member with the same condition.
Research on the entire human genome (or all the genes in a human being) has found that certain genes related to human leukocyte antigen (HLA – proteins in our bodies that help the immune system distinguish the body’s own proteins from proteins made by foreign invaders like viruses and bacteria) might contribute to the development of Alopecia Areata. Some variations in these HLA genes, specifically HLA-DRB1*04 and HLA-DRB1*16, can make people more likely to get the condition. HLA-DQB1*03, another variant, has also been linked to Alopecia Areata. It’s also worth noting that certain gene variations are even more strongly linked to AT and Alopecia Universalis (AU – condition that causes complete hair loss on the entire body), rather than Alopecia Frontalis (AF – condition that causes hair loss at the front of the scalp). These include HLA-DRB1*1104 and HLA-DQB1*0301.
However, it’s not just about genes. Environmental factors like sickness, infection, medication usage, and stress might also play a part in triggering episodes or causing the disease to come back after a period of remission.
Risk Factors and Frequency for Alopecia Totalis
The chances of developing acute appendicitis (AA) in one’s lifetime are about 2%, while acute thrombosis (AT) is less common, affecting only around 0.03% of people. However, there has been a rise in AT cases recently. For those who have atrial fibrillation (AF), less than 10% will develop AT. Though children and young adults are most frequently affected, AT can occur at any age. Both males and females can develop the disease at the same extent.
Signs and Symptoms of Alopecia Totalis
When a patient comes in with significant hair loss, it’s important for doctors to take a detailed history. People who have Alopecia Totalis (AT), a condition that results in total hair loss from the scalp, often begin with patchy hair loss that gradually worsens. Only 5%-10% of these patients progress to complete or near-complete scalp hair loss. The usual course for this condition typically begins with more than 5 separate patches of hair loss which ultimately progress to AT usually within 1 year, and within 4 years for 90% of patients.
Family history and past health conditions are crucial to understanding the patient’s situation. Approximately 20% of cases have a family history of hair loss. Also, patients with Alopecia Areata (AA), the condition that often precedes AT, frequently have other health problems. About 20% of these patients experience additional conditions, most commonly autoimmune thyroid disease, vitiligo, type 1 diabetes, atopy, and inflammatory bowel disease.
Upon medical examination, almost all scalp hair is gone in patients with AT, but hair in other areas like eyelashes, eyebrows, armpits, and pubic area is usually preserved. In contrast to scarring alopecia, where hair follicles disappear, you can still see hair follicles on the scalp in AT patients. These patients usually don’t have much redness or scaling on their scalp. You might also spot ‘exclamation point hairs’ – thick at the top and thin at the bottom – at the edges of the hairless patches. If a patient’s hair begins to grow back spontaneously, it might initially lack pigment, but this usually comes back over a few weeks or months.
Nails are another important area to check, as they can be affected in alopecia areata, particularly in cases that have progressed to AT. This is seen in about 20% of patients. The most common nail issue is nail pitting, but fragile nails, rough nails, and separation of the nail from the nail bed can also occur.
Testing for Alopecia Totalis
The diagnosis of certain conditions is often possible with just your history and a physical examination by the doctor. For example, if you’re losing hair in patches and this advances to total loss of hair on your scalp, it’s often a clear signal of your condition. In an examination, your doctor might use a dermatoscope, a special skin-viewing instrument. When examining hair specifically, this is called trichoscopy. It can reveal details like small yellow dots around hair follicles, black dots, thin hairs and short, fine hairs.
If your doctor isn’t quite sure about your condition, a biopsy, or small sample of your scalp, might be taken for further examination. Usually, one or two small sections of the scalp will be removed with an instrument called a punch. These samples can be studied in different ways by cutting them in different directions, either horizontally or vertically. Sometimes it may be necessary to take more than one punch biopsy, to examine the hair follicles comprehensively.
In some cases, your doctor may need to run some lab tests. These might test for issues with your thyroid, using a thyroid-stimulating hormone (TSH) test that evaluates the level of a hormone that can influence your thyroid function. Other tests, like a complete blood count (which gives a broad look at your overall health), are employed in general to determine the levels of various vitamins and minerals in your body. Remainder tests might look for specific diseases, like syphilis using a rapid plasma reagin (RPR) test, or evaluate the levels of certain hormones in your blood, such as testosterone and dehydroepiandrosterone sulfate (DHEA-S). An antinuclear antibody (ANA) test also might be ordered to check if your immune system is attacking your own body.
Treatment Options for Alopecia Totalis
There are quite a few ways to treat alopecia areata (AA), which could be localized or could affect the whole scalp (alopecia totalis, or AT). It can be treated through directly injecting medicine into the affected area or through topically applied or oral medicines. The most successful treatments found so far include topical immunotherapy, light treatments, systemic corticosteroids, and immunosuppressive medicines. There have also been successful results with JAK inhibitors and statins mixed with cholesterol lowering medicines in individual cases, although tests on larger scale are still needed.
Topical Steroids
Topical steroids are often the first line of treatment for alopecia totalis. These are generally used in children under 10 years old who have widespread hair loss. High-potency topical steroids like clobetasol 0.05% solution or foam are often recommended. Children who have had the condition for less than a year tend to respond better than those with a longer duration.
Topical Immunotherapy
Topical immunotherapy is used when alopecia areata is severe. This involves using chemicals such as diphenylcyclopropenone (DPCP) or squaric acid dibutyl ester (SADBE) to cause an allergic reaction that encourages hair growth. The exact way this works isn’t fully understood. These treatments are typically applied weekly and the most common side effects include local irritation, itchiness, or rashes.
Systemic Steroids
Systemic (whole-body) steroids are also commonly used to stimulate hair regrowth with good results. Monthly injections or oral steroids have shown the most success. Potential side effects of systemic steroid treatment include weight gain, acne, and disruption in menstrual cycles. Unfortunately, hair loss often recurs once the treatment is discontinued.
Immunosuppressive Agents
If the initial treatments aren’t successful, medications that suppress the immune system can be considered. These include cyclosporine, methotrexate, or azathioprine, either alone or with systemic steroids. These medications are often given for 6-12 months.
JAK Inhibitors
JAK inhibitors are a newer type of medication currently being studied for treating alopecia areata. These agents have shown promising results in treating other inflammatory conditions such as rheumatoid arthritis and psoriasis. The main side effects include increased risk of infections, liver problems, and blood count decreases.
Phototherapy
Light therapy (phototherapy) and photodynamic therapy (PDT) have been used with reported success in cases of extensive alopecia areata. Adverse effects of light therapy include skin cancer, hence this treatment should be used cautiously.
Simvastatin
In a few individual cases, daily simvastatin administered alongside ezetimibe, a cholesterol-lowering medication, showed successful treatment outcomes.
Nonmedical Interventions
In conjunction with medical treatments, items that conceal hair loss like wigs, hairpieces, or scalp prostheses can offer immediate coverage while other treatments take effect. They can be purchased ready-made or customized. Due to the visible nature of hair loss and its significant emotional impact, many patients choose to use these products while undergoing medical treatments.
What else can Alopecia Totalis be?
When trying to diagnose Alopecia Totalis (AT), doctors need to look at other conditions that could be causing the hair loss. These include:
- Telogen effluvium, a type of temporary hair loss
- Anagen effluvium, another form of temporary hair loss
- Androgenetic alopecia, also known as male or female pattern baldness
What to expect with Alopecia Totalis
AT, a severe type of alopecia areata (AA), has a poorer outlook compared to the more common form of AA. Almost a quarter of patients with AT don’t respond to any treatment. Initially, recovery rates may seem high, but they often drop in the long-term due to the condition’s likelihood to return.
Spontaneous recovery, where the condition improves on its own, happens in less than 10% of patients. Moreover, only about 8.5% of patients fully recover in the long-term. When assessing the prognosis, having a family history of AA and the severity of AA at the start can be considered as risk factors for a poor outcome.
Interestingly, there’s a recently identified type of AT called acute diffuse and total alopecia (ADTA) that mostly affects young adults. This form of AT generally has a better outlook than other types.
Possible Complications When Diagnosed with Alopecia Totalis
People with AA often experience mental health issues, including anxiety, phobias, depression, and panic disorders. The severity of these conditions is usually proportional to the seriousness of AA. Hence, individuals with AT are even more likely to experience these mental health troubles due to their associated health condition.
Common mental health issues include:
- Anxiety
- Phobias
- Depression
- Panic disorders
Preventing Alopecia Totalis
Patients should understand that this condition is long-lasting and may require ongoing management. Before starting treatment, it’s important to talk about the different options available, any possible side effects, and the chances of the disease coming back. It’s also important to understand that this condition can cause significant emotional stress, and it’s okay to seek help from mental health professionals when needed.
Also, there are organizations specifically dedicated to this condition, like the National Alopecia Areata Foundation (NAAF). These organizations provide more information about the disease and offer a platform to connect with other people going through the same experience.
