What is Monilethrix?

Originally identified in 1897 by Walter Smith, Monilethrix is a relatively rare hair condition that leads to brittle hair and patchy hair loss. People usually inherit this condition from their parents as it mostly passes from one generation to the next. In this condition, the hair shaft, or the part of the hair that we can see, becomes periodically thin, creating a bead-like appearance. The word ‘Monilethrix’ actually gets its name from the Latin word ‘monile’ meaning ‘necklace’ and the Greek word ‘thrix’ meaning ‘hair’. This is because the hair of a person with Monilethrix looks like a string of beads or a necklace.

What Causes Monilethrix?

Monilethrix is a genetic hair condition that is often passed down through families. It involves a genetic mutation or change that mostly affects the hair’s protein structure, leading to hair that is weak and brittle. These changes occur in genes known as KRT 81, KRT 83, KRT 86, which are vital for healthy hair growth. These genes are located on a specific area of our DNA called chr12q13 and are basically the instructions for creating specific proteins needed for hair, called hHb6 and hHb1.

There is also another, less common, type of Monilethrix that is inherited in a slightly different way and usually doesn’t show the same regular pattern of weak spots in the hair. This form of Monilethrix is thought to result from changes in another gene called the desmoglein 4 gene (DSG4). This gene is important because it’s the only one of its type that is ‘expressed’ or ‘turned on’ in the part of the hair that becomes hard and gives the hair its structure.

Risk Factors and Frequency for Monilethrix

Monilethrix, a condition affecting the hair, doesn’t seem to favour any specific race, sex, or hair color. The exact number of people who have or develop the condition is unknown.

Signs and Symptoms of Monilethrix

Monilethrix is a hair condition that usually starts in early childhood. When children are born, their hair appears to be perfectly normal, but after several months they begin to show signs of having hair that’s short and prone to breaking. As a result, their hair doesn’t grow long enough to need a haircut.

The severity of the condition varies from person to person. Some people might have a scalp that looks almost normal with only a few affected hair follicles, while others could experience total hair loss. The tell-tale sign is when the hair on the scalp is unusually thin all over, and extremely short and fragile. This breakable hair is particularly prevalent in areas commonly exposed to friction, like the top and back of the head.

In more severe cases of monilethrix, other hairy areas such as eyelashes, eyebrows, and body hair can be affected too.

This hair condition is often paired with other symptoms relating to the hair follicles. These can range from redness around the hair follicle to larger, hard bumps on the scalp, most often seen at the back of the head. The weak hair may also come with nodules, a condition called trichorrhexis nodosa, or other rare skin-related symptoms.

  • Syndactyly (fused fingers or toes)
  • Cataracts (cloudiness in the lens of the eye)
  • Dental abnormalities
  • Nail abnormalities, such as koilonychia (spoon-shaped nails) or brittle nails

Testing for Monilethrix

To diagnose monilethrix, a rare hair disorder, doctors mostly rely on your medical history and a physical examination. They usually confirm the diagnosis by examining your hair under a microscope or using a device known as a trichoscope.

Microscopic examination of the hair in people with monilethrix typically reveals a pattern that looks like beads on a string, with segments of the hair alternately appearing squeezed and normal. These different segments are usually about 0.7 to 1.0 mm apart.

Trichoscopy is another method that doctors use. It’s simple, non-invasive (doesn’t require any cuts or needles), and takes less time. This technique allows doctors to identify monilethrix by showing a significantly higher percentage of anagen hair (the growth phase during which hairs are actively growing), and a key characteristic: a “necklace” appearance of the hair.

When your hair is viewed under high magnification using trichoscopy, the doctors see regular variations in the diameter of your hair. It will show alternating wide (nodes) and narrow (internodes) sections, which make your hair fragile at those points. The wider sections will appear normal and contain the hair’s medulla (innermost layer), while the narrow sections usually do not have a medulla. This pattern confirms the presence of monilethrix.

Treatment Options for Monilethrix

So far, there is no cure for this hair condition. However, various treatments like applying minoxidil to the scalp, taking oral medications like acitretin or griseofulvin, or using systemic corticosteroids and peeling ointments have shown benefits in some cases. Low dose oral minoxidil appears to be a promising and well-tolerated treatment. N-acetyl cysteine, another medication, has been used in some cases but symptoms have come back after initial improvement.

For cosmetic reasons, wigs can be considered, but it’s important to note that the friction caused by wig adhesives might make the hair loss worse.

The best way to manage this hair condition is to avoid things that can cause chemical and mechanical damage. This includes excessive hair combing or washing and any kind of friction. This is why it’s important for the person with this condition and their parents to be informed about lifestyle changes they may need to make.

When examining a case of monilethrix, a hair shaft disorder, doctors also need to consider other conditions that could possibly explain the symptoms. These may include:

  • Pseudo-monilethrix
  • Congenital alopecia (birth-defect baldness)
  • Trichorrhexis invaginata (another hair shaft defect)
  • Ectodermal dysplasia (a group of disorders affecting the skin, hair, nails, and sweat glands)

What to expect with Monilethrix

The outcome for people with a hair condition called monilethrix is different for each person. For some, the condition improves on its own when they become adults, but for many, it lasts their entire life. There have been observations that the hair may look healthier and grow back seemingly normal during summer, during pregnancy, and around puberty.

It has also been suggested that hormones could play a part in the improvement of the condition. For instance, there was a report of a patient’s condition improving after her first period.

Possible Complications When Diagnosed with Monilethrix

Monilethrix, a hair disorder, can often cause patients to experience low self-esteem and a negative self-image. This emphasizes the importance of evaluating its effects on a person’s overall quality of life. Besides these psychological effects, treatment methods for this condition can also lead to complications.

Key Points:

  • Monilethrix can lead to low self-esteem and negative self-perception.
  • It’s vital to assess the effect of this condition on a patient’s quality of life.
  • There can be complications related to the treatments for Monilethrix.
Frequently asked questions

Monilethrix is a relatively rare hair condition that leads to brittle hair and patchy hair loss.

The exact number of people who have or develop the condition is unknown.

The signs and symptoms of Monilethrix include: - Hair that is short and prone to breaking, leading to hair that doesn't grow long enough to require a haircut. - Unusually thin and fragile hair on the scalp, particularly in areas exposed to friction like the top and back of the head. - In more severe cases, other areas of hair such as eyelashes, eyebrows, and body hair can also be affected. - Additional symptoms related to the hair follicles, such as redness around the hair follicle, hard bumps on the scalp (often at the back of the head), and nodules. - Monilethrix can also be associated with other conditions or abnormalities, including syndactyly (fused fingers or toes), cataracts (cloudiness in the lens of the eye), dental abnormalities, and nail abnormalities such as spoon-shaped nails or brittle nails.

Monilethrix is a genetic hair condition that is often passed down through families. It involves a genetic mutation or change that affects the hair's protein structure.

The other conditions that a doctor needs to rule out when diagnosing Monilethrix are: - Pseudo-monilethrix - Congenital alopecia (birth-defect baldness) - Trichorrhexis invaginata (another hair shaft defect) - Ectodermal dysplasia (a group of disorders affecting the skin, hair, nails, and sweat glands)

The types of tests needed for Monilethrix include: 1. Microscopic examination of the hair: This involves examining the hair under a microscope to look for a pattern that resembles beads on a string, with alternating segments of squeezed and normal hair. 2. Trichoscopy: This non-invasive technique allows doctors to identify Monilethrix by observing regular variations in the diameter of the hair. It shows alternating wide (nodes) and narrow (internodes) sections, confirming the presence of Monilethrix. These tests help doctors confirm the diagnosis of Monilethrix and differentiate it from other hair disorders.

Monilethrix can be treated with various methods such as applying minoxidil to the scalp, taking oral medications like acitretin or griseofulvin, or using systemic corticosteroids and peeling ointments. Low dose oral minoxidil has shown promise as a well-tolerated treatment. N-acetyl cysteine has also been used in some cases, although symptoms may return after initial improvement. Additionally, for cosmetic reasons, wigs can be considered, but it's important to note that wig adhesives may worsen hair loss. Managing Monilethrix involves avoiding chemical and mechanical damage, such as excessive hair combing or washing and any kind of friction. Lifestyle changes may be necessary for individuals with this condition and their parents to prevent further damage.

There can be complications related to the treatments for Monilethrix.

The prognosis for Monilethrix varies for each person. For some, the condition improves on its own when they become adults, but for many, it lasts their entire life. There have been observations that the hair may look healthier and grow back seemingly normal during summer, during pregnancy, and around puberty. It has also been suggested that hormones could play a part in the improvement of the condition.

Dermatologist.

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