Understand congenital hypotrichosis and its characteristics with Meri Sehat. Gain insights for informed decisions and care.

Congenital Hypotrichosis

Congenital hypotrichosis is described as being born with less hair than normal<a href="#accordion">[1]</a>. 
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Introduction

Unlike alopecia where there&rsquo;s hair loss, hypotrichosis is a condition where there&rsquo;s no hair growth. In congenital hypotrichosis, babies are born with less hair than would appear to be normal at birth. However, in newborns, the amount of hair can range between extremes and still be considered normal.

Congenital hypotrichosis usually results from a genetic mutation, or abnormality during embryonic development. Worldwide, 1 in 10,000 individuals suffers from the disease<a href="#accordion">[2]</a>. 
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Understanding  Congenital Hypotrichosis

Some common types of congenital hypotrichosis are:

<ul>
	<li>Congenital Aplasia (Aplasia Cutis Congenita) - This is an embryonic developmental defect where the skin fails to form. The baby is born with a skin patch resembling an ulcer or open wound, often at the center of a &lsquo;whorl pattern&rsquo; of hair growth. Over time, the skin may scab and form a scar. However, in the case of a large patch, surgery may be needed to close the wound.</li>
	<li>Triangular Alopecia - It is quite similar to congenital aplasia, except it occurs in a triangular patch of skin over the temple. Hair follicles in that triangular patch may fail to grow. However, unlike congenital aplasia, there are no open ulcers on the patch. Doctors often transplant the donor&rsquo;s hair follicles onto the affected area.</li>
	<li>Congenital Atrichia - Also called papular atrichia, this condition is characterized by an absence of a patch. The child usually loses all of their hair early in childhood, and the hair never grows back. This occurs due to the hair follicles entering the telogen phase in the early stages of childhood. Researchers believe it results from a single gene defect. 
	&nbsp;</li>
</ul>

Depending on its distribution, congenital hypotrichosis can be classified as regional or general. 
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Typically, children are born with no sign of hair growth, not only on the scalp but also on the eyebrows or skin. In some cases, the child experiences unusual hair fall within one year of birth. Moreover, in cases of congenital aplasia, the baby will have open ulcers or wounds on the scalp. However, in some cases, children are born with short, fine hair at birth.

Other common signs and symptoms of congenital hypotrichosis are:

<ul>
	<li>Keratosis Pilaris</li>
	<li>Pruritus of the scalp</li>
	<li>Scarring of the scalp</li>
	<li>Loss of hair all over the body 
	&nbsp;</li>
</ul>

Furthermore, due to the loss of hair, children are more susceptible to infections and sunburn. 
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Several other conditions can also cause hypotrichosis. These include:

<ul>
	<li>Marie Unna hypotrichosis</li>
	<li>Graham-Little Syndrome</li>
	<li>Ofuji Syndrome</li>
	<li>Cartilage-hair hypoplasia</li>
	<li>Jeanselme and Rime Hypotrichosis</li>
	<li>Metaphyseal Chondrodysplasia</li>
	<li>Scalp nevi 
	&nbsp;</li>
</ul>

Based on the association with other conditions, children may be born with the following features:

<ul>
	<li>Skin, teeth, and nails abnormality</li>
	<li>Deafness</li>
	<li><a href="https://merisehat.pk/disease/epilepsy">Epilepsy</a></li>
	<li>Errors of Metabolism</li>
	<li>Skeletal abnormalities 
	&nbsp;</li>
</ul>

Hair loss not only affects an individual on a physical level but also psychologically. Children with congenital hypotrichosis often experience mental health issues due to insecurity regarding their appearance. 
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Congenital hypotrichosis results from genetic abnormalities or defects during embryonic development. These abnormalities interfere with the functioning of hair follicles.

A normal hair growth cycle has three phases: the growing phase (anagen), the regressing phase (catagen), and the resting phase (telogen). In congenital hypotrichosis, the hair follicles enter the telogen stage early in childhood. This disrupts the communication between the modified keratinocytes and fibroblasts. The lack of communication does not let a new anagen phase start. Thus, the hair is unable to grow. 
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There are no specific screening tests for congenital hypotrichosis. Doctors usually rely on a child&rsquo;s clinical presentation to confirm their diagnosis. 
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The diagnosis of congenital hypotrichosis depends on the clinical history and physical examination. At the time of delivery, the child may lack hair on the scalp, eyebrows, and skin. However, in some cases, the child may experience unusual hair fall within the first year. In congenital aplasia, a patch with ulcers of wounds will be present on the baby&rsquo;s scalp. 

Doctors may also conduct hair microscopy to identify the stages of the growth cycle for the hair samples. 
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Since it results from a genetic alteration, there is no cure for congenital hypotrichosis. However, in some cases, such as triangular alopecia and congenital aplasia, the abnormal patch may be surgically removed and implanted with hair follicles from a healthy donor. 
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The permanent loss of hair can predispose one to infections and sunburn. 
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Congenital hypotrichosis is a genetically transmitted disease, so any family history can make the offspring susceptible to this disease. However, many cases occur without any family history of the condition. 
 
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Conclusion

Since congenital hypotrichosis results from genetic abnormalities, there are no known preventive measures. Parents with children suffering from congenital hypotrichosis can be referred for genetic counseling to determine their risk of passing it on to further offspring. Moreover, it can help determine whether hypotrichosis is part of a syndrome.&nbsp; 
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Key takeaways

<ul>
	<li>Congenital hypotrichosis is characterized by congenital hair follicle deficiency. Children are born with less hair than normal. However, the amount of hair in a newborn child can vary between extremes and still be considered normal early in life.</li>
	<li>Unlike alopecia where there&rsquo;s hair loss, hypotrichosis is a condition where there&rsquo;s no hair growth.</li>
	<li>Congenital hypotrichosis usually results from a genetic mutation or abnormality during embryonic development.</li>
	<li>Typically, children are born with no sign of hair growth, not only on the scalp but also on the eyebrows or skin. In some cases, the child experiences unusual hair fall within one year of birth. Moreover, in cases of congenital aplasia, the baby will have open ulcers or wounds on the scalp.</li>
	<li>The permanent loss of hair can predispose one to infections and sunburn.</li>
	<li>There are currently no treatments available for congenital hypotrichosis.</li>
</ul>

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