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What Is Benign Hereditary Telangiectasia?

An inherited disorder called benign hereditary telangiectasia results in dilated blood vessels on the skin and lips. It doesn't cause any systemic symptoms, in contrast to other illnesses of a similar nature.

A rare disorder known as benign hereditary telangiectasia (BHT) results in the development of numerous tiny, dilated blood vessels across your skin, accompanied by colorful blemishes.

There are many different forms of telangiectasia, and while many of these "spider veins" grow with age and sun exposure, BHT is a rare variant that can appear in early childhood. Experts believe that there might be a genetic component as a result.

BHT is not a sickness of the system. However since a lot of the symptoms resemble those of more serious illnesses, a proper diagnosis from a physician is crucial.

Learn more about BHT, its causes, symptoms, and available treatments by continuing to read.

How does BHT differ from hereditary hemorrhagic telangiectasia?

To distinguish it from a more dangerous disorder known as hereditary hemorrhagic telangiectasia (HHT), BHT is also occasionally referred to as hereditary benign telangiectasia.

Previously known as Osler-Weber-Rendu syndrome, hereditary hemolytic anemia (HHT) results in vascular abnormalities all over the body. HHT is a systemic disorder as opposed to BHT.

While BHT symptoms are limited to the skin, HHT can also have an impact on internal organs and mucous membranes. These consist of your liver, lungs, and brain.

Also, BHT usually doesn't result in bleeding, in contrast to HHT. Life-threatening bleeding is a potential side effect of HHT, especially if it happens in the brain.

A nosebleed that occurs on its own and repeatedly is one of the early indicators of HHT. This typically manifests before telangiectasia on the skin and mucous tissues.

Pulmonary hypertension and immune system malfunction are potential side effects of HHT. But aside from age-related skin lesions, BHT doesn't seem to be linked to any health issues.

What are the symptoms of benign hereditary telangiectasia?

Other than cosmetic symptoms, BHT usually produces none. This is not the same as HHT or other illnesses that could result in more widespread symptoms.

Clinical indicators may be seen on your lips and skin by you and your physician. BHT typically manifests as oval or circular lesions with underlying dilated blood vessels.

BHT generally causes:
  • spider veins that might start on your legs and move to your arms and trunk
  • smaller, dilated veins that could have halos of a lighter hue surrounding them
  • unusual vein enlargement near the lips, but no bleeding
  • dilated veins that soften with age and can be either red or purple, depending on skin tone
  • dilated veins that enlarge with aging
  • veins that are impacted but do not exhibit any other symptoms, such as burning, bleeding, or stinging

What causes benign hereditary telangiectasia?

BHT's precise cause is unknown. Nonetheless, specialists believe that family history and genetics might be important. Since there is no recognized etiology for this illness, it is referred to as "idiopathic."

BHT typically manifests clinical symptoms in children between the ages of two and twelve, which further points to a genetic component. BHT symptoms can occasionally be seen from birth.

A 2017 case study, however, talked about the development of BHT in a female who had no known familial history of the condition. This also points to the possibility of this syndrome developing sporadically.

There are additional risk factors for BHT in other forms of telangiectasias as well. Among them are:
  • pregnancy
  • being female
  • sun exposure

How is benign hereditary telangiectasia inherited?

According to experts, BHT is inherited in an autosomal dominant manner. This indicates that each child has a 50% probability of inheriting it from their parent.

On the other hand, nothing is known about the precise genetics of BHT. Clinical research is required to ascertain whether BHT can develop without a family history and what impact any genetic alterations might have.

How do doctors diagnose benign hereditary telangiectasia?

BHT can be diagnosed by a physical examination. In addition to identifying the outward manifestations of BHT, a dermatologist might inquire about any known family history of the condition.

Removing the possibility of additional telangiectasias is another step in the diagnosis of BHT. A dermatologist will thus search for more severe indications, like bleeding and dilated veins along your mucosal membranes.

To assist in distinguishing BHT from other vascular or systemic disorders, a doctor could additionally prescribe a skin biopsy.

What is the treatment for benign hereditary telangiectasia?

For BHT, treatment is not medically required. For cosmetic purposes, some people might like to lessen the visibility of their dilated veins.

A dermatologist may suggest one of the following in such circumstances:
  • Electrosurgery: Electrosurgery was initially created in the 1920s as a method to halt bleeding, and it is currently widely utilized in many dermatological contexts. Eliminating lesions with heat produced by electromagnetic fields might benefit BHT.
  • Intense pulsed light (IPL) therapy: Using polychromatic light waves, IPL can help treat BHT and eliminate spider veins.
  • Sclerotherapy: Sclerosing injections are used by medical practitioners to treat a variety of vascular diseases.
  • Vascular laser therapy: Laser therapy is an additional option for eliminating venous irregularities. This treatment targets and destroys blood vessels using heat. The American Society for Dermatologic Surgery states that this is the recommended procedure for treating facial veins.
A dermatologist may prescribe BHT for its cosmetic effects, but they will also want you to keep an eye out for any changes in your skin.

Another way to lessen the appearance of BHT lesions is to wear sunscreen. This is particularly crucial for areas of your body like your hands, chest, and face that are frequently exposed to the sun.

What is the outlook for people with benign hereditary telangiectasia?

All things considered, BHT has a bright future. BHT does not result in any medical issues, in contrast to other telangiectasias like HHT that could have greater systemic implications.

To rule out more serious illnesses, a doctor must examine any suspected signs of BHT in a child.

When you become older, lesions in your upper body may also start to form new ones and change in color and shape.

Takeaway

BHT is not a dangerous illness. However, a physician must distinguish BHT from other telangiectasias, such as HHT, or systemic disorders, which may have more detrimental effects on health.

Furthermore, there is currently no proven method for preventing BHT. Although BHT doesn't need to be treated, some people choose to do so if a lesion affects them cosmetically.



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