Alopecia Totalis

Understanding Alopecia Totalis: Causes, Symptoms, and Treatment Options

Alopecia totalis is a more advanced form of alopecia areata, and results in complete loss of hair on the scalp. It’s an autoimmune condition and occurs when the immune system mistakenly targets hair follicles. Unlike alopecia areata, which results in localised patches of hair loss, alopecia totalis results in loss of all scalp hair. This condition often starts as patchy hair loss (alopecia areata), before progressing to total scalp baldness. Although it does not cause physical pain or illness, its impact to appearance can have significant self-esteem impacts.

Profile of women showing showing alopecia totalis hair loss

What is Alopecia Totalis

Alopecia totalis is an autoimmune condition, an advanced form of alopecia areata, that results in complete scalp baldness. Unlike the patchy hair loss seen in earlier stages of alopecia areata, alopecia totalis causes widespread follicle damage. This occurs because the immune system mistakenly attacks hair follicles, disrupting their normal growth cycle and halting hair production. This hair loss is typically sudden and occurs rapidly. The exact causes are not fully known, but it is believed to result from a combination of genetic predisposition and immune system dysfunction.

Causes of Alopecia Totalis

Alopecia totalis is a complex autoimmune condition, with no single cause. While the underlying causes are not known, research indicates a combination of genetic predisposition, immune system dysfunction, and environmental factors.

Autoimmune Dysfunction

  • Alopecia totalis is primarily caused by an autoimmune response, where the body’s immune system mistakenly identifies hair follicles as harmful and attacks them. This immune response disrupts the natural hair growth cycle, causing the follicles to shrink and eventually stop producing hairs completely. The progression from alopecia areata to totalis indicates a more severe and sustained autoimmune attack.

Genetics

  • Genetics play a significant role, as those with a family history of immune disorders are at a higher risk. Researchers have identified specific genes, particularly in the HLA (human leukocyte antigen) region, that may increase susceptibility. These genes affect how the immune system distinguishes between healthy cells and perceived threats.

Other Autoimmune Conditions

  • Alopecia totalis often co-exists with other autoimmune disorders, such as thyroid disease, vitiligo, or lupus. This association between conditions suggests a shared underlying dysfunction in the immune system. Those with multiple autoimmune conditions may have an increased risk of developing more severe forms of alopecia.

Hormonal and Biological Factors

  • While alopecia totalis is not directly caused by hormonal changes, fluctuations in hormones may trigger or exacerbate this condition. Changes in hormones, such as stress or puberty, can influence the immune system activity. Biological factors, such as the activity of cytokines and other immune signaling molecules, can also be significant. Cytokines are proteins that help the immune system communicate and coordinate its response. In those with alopecia totalis, certain cytokines, including interferon-gamma and tumor necrosis factor-alpha (TNF-α), are overproduced. These cytokines contribute to the immune system’s attack on hair follicles, preventing them from producing new hair. Additionally, an imbalance in regulatory T-cells, which are responsible for suppressing overactive immune responses, may allow this condition to escalate.

Environmental Triggers

  • In those that are genetically predisposed, environmental factors can act as a catalyst for developing or progressing alopecia totalis. Potential triggers can include viral infections, significant physical or emotional stress, or exposure to certain toxins. These factors can activate or exacerbate the immune system’s attack on hair follicles, leading to extensive hair loss.

Symptoms of Alopecia Totalis

The main symptom of alopecia totalis is complete loss of hair, but the symptoms can extend beyond visible hair loss. As a more severe form of alopecia areata, it involves extensive immune system activity that can impact other parts of the body.

Complete Scalp Hair Loss

  • The most significant symptom is the total loss of hair on the scalp. This can occur gradually, with patches of hair loss merging over time, or happen more rapidly in weeks or months. The scalp will usually appear smooth and free of scarring, as the hair follicles are not permanently destroyed.

Scalp Sensations

  • Some experiences sensations such as itching, tingling, or burning on the scalp before or during hair loss. It’s believed that these are a result of the inflammation in the hair follicles caused by the immune system’s attack. While not everyone experiences these symptoms, it can serve as an early warning of impending hair loss.

Loss of Eyebrows and Eyelashes

  • Although it primarily affects the scalp, some may experience losing their eyebrows and eyelashes. Along with the hair loss, this can have a significant impact on overall appearance, and self-esteem.  

Nail Changes

  • Approximately 10-20% of people with alopecia totalis experience changes in their fingernails and toenails. Common nail changes include pitting (small dents in the nail surface), ridging, thinning, or brittleness. In some cases, the nails may develop a rough texture or white spots, which can help with diagnosing the condition.

Treatment Options for Alopecia Totalis

While there is no cure for alopecia totalis, there are treatments available to manage this condition and encourage hair growth. Treatments often combine supressing the immune system and stimulating hair follicles. Effectiveness can vary, with some experiencing significant regrowth while others have limited success.

Corticosteroids

  • Corticosteroids are commonly used to suppress the overactive immune response responsible for attacking hair follicles. Corticosteroids help reduce inflammation in the scalp, allowing hair follicles to resume their normal function. They can be administered in various forms:

      • Injectable Corticosteroids: Injected directly into affected areas of the scalp. These are often the first treatment option. 
      • Topical Corticosteroids: Applied as creams or lotions, though their effectiveness may be less pronounced compared to other forms.
      • Oral Corticosteroids: These are often reserved for severe cases due to the potential systemic side effects.

Topical Immunotherapy

  • Topical immunotherapy involves applying chemicals such as diphencyprone (DPCP) or squaric acid dibutylester (SADBE) to the scalp. These substances cause a mild allergic reaction, which distracts the immune system and prevents it from targeting hair follicles. Over time, this process may reduce hair follicle damage and promote regrowth. Topical immunotherapy is often used for persistent or severe cases of alopecia totalis.

Janus Kinase (JAK) Inhibitors

  • Janus kinase (JAK) inhibitors, such as tofacitinib and ruxolitinib, are drugs that target the immune pathways involved in alopecia totalis. These drugs block the signals that cause inflammation and immune attacks on hair follicles. Clinical trials have showed positive results with significant regrowth in some cases.

Anthralin (Dritho-Scalp)

  • Anthralin is a topical treatment originally developed to treat psoriasis but is often used for alopecia totalis. It works by modulating the immune response and inducing mild scalp irritation, which can stimulate hair regrowth. Anthralin is typically applied to the scalp for a short period and then washed off to minimize side effects.

Minoxidil (Rogaine)

  • Minoxidil is a topical medication that stimulates hair follicles and increases blood flow to the scalp. While it is primarily used for androgenetic alopecia, it may help by encouraging the regrowth of fine, vellus hair. Minoxidil is often combined with other treatments to enhance its effectiveness.

Platelet-Rich Plasma (PRP) Therapy

  • PRP therapy involves extracting a small amount of blood, processing it to concentrate platelets, and injecting it into the scalp. The growth factors in platelets can help stimulate hair follicle activity and repair damaged tissue. While PRP therapy is more commonly used for other forms of hair loss, it has shown positive results as a complementary treatment.

Low-Level Laser Therapy (LLLT)

  • LLLT uses low-intensity laser light to stimulate hair follicles and improve blood flow to the scalp. While its efficacy for alopecia totalis is not as well documented as other forms of hair loss, some have reported positive results. LLLT is non-invasive and can be used as a supplementary treatment.

Frequently Asked Questions (FAQ)​

Alopecia totalis is an advanced form of alopecia areata characterized by the complete loss of hair on the scalp. It is considered an autoimmune condition, where the immune system mistakenly attacks hair follicles, leading to hair loss.

The exact cause of alopecia totalis is unknown, but it is believed to be triggered by a combination of genetic, environmental, and autoimmune factors. Stress, certain infections, or other autoimmune conditions may act as potential triggers.

Alopecia totalis can be unpredictable. In some cases, hair regrowth occurs spontaneously, while in others, the condition can persist for years or even become permanent. Treatment options aim to stimulate regrowth, but their effectiveness varies by individual.

Treatment options include corticosteroid injections or creams, topical immunotherapy, oral immunosuppressants, and JAK inhibitors. While these treatments may help stimulate hair regrowth, their success is not guaranteed, and they may not work for everyone.

Alopecia totalis specifically refers to complete hair loss on the scalp. If hair loss extends to other areas of the body, including eyebrows, eyelashes, and body hair, the condition is classified as alopecia universalis. Both conditions share similar underlying mechanisms but differ in the extent of hair loss.

Conclusion

Alopecia totalis can be a challenging autoimmune condition that results in complete hair loss, having significant emotional impact. While the exact cause is not fully understood, it’s a combination of genetic predisposition, and immune system dysfunction. Though there is no cure, there are a variety of treatments to help manage and even promote hair regrowth.

Want to Learn More?

View the latest scientific paper on Totalis Alopecia from the National Library of Medicine.

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