This life-threatening disease has all the signs we’ve come to associate with vampires – Firstpost

Sharp fangs, pale skin and allergy to sunlight - if you think we are talking about the vampires from Bram Stokers immortal novel, you are mistaken!

We are, however, talking about a disease that takes its name from Dracula, Edward Cullen, Eric Northman, and Damon and Stefan Salvatore - and other famous vampires whose legend spans books, theatre, TV and cinema.

The condition that resembles all the symptoms weve come to associate with Dracula and his hoard, is called the vampire disease - medically known as Hypohidrotic ectodermal dysplasia.

Model Brooks Ginnan has Ectodermal Dysplasia, also known as the vampire disease. Image source: Getty Images.

Hypohidrotic ectodermal dysplasia is a common form of ectodermal dysplasia. According to one estimate, it occurs in one in 10,000 newborns worldwide. (Ectodermal refers to the outermost layer in the embryo that goes into making the skin, hair, teeth and sweat glands, etc.; and dysplasia is an abnormal growth. Hypohidrosis means a condition in which one sweats too little.)

Hypohidrotic ectodermal dysplasia is a rare genetic condition linked with the X chromosome. It is more prevalent in males as they have only one X chromosome in their DNA.

The cause: a variation in specific genes EDAR, EDARADD, and WNT10A that tell the body to make proteins that are needed early in life to make several organs.

Changes in these genes lead to deformities of structures like the skin, hair, nails, sweat glands and teeth - all of them develop from the same ectoderm or primary layer of a growing embryo.

A parent may pass the faulty gene on to a child or the genes can mutate in a child without either parent having a faulty gene.

People with Hypohidrotic ectodermal dysplasia present with a triad of signs: sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth (anodontia or hypodontia), and inability to sweat due to the lack of sweat glands (anhidrosis or hypohidrosis).

Other prominent symptoms of this condition are scanty or no eyebrows and eyelashes with bulging of the forehead (frontal bossing).

People living with Hypohidrotic ectodermal dysplasia also tend to have darkening or hyperpigmentation around the mouth (perioral) and eyes (periorbital) - the latter may look like dark circles. The skin over these areas is usually dry and wrinkled.

Those suffering from this condition may have a depressed nasal bridge, clogged nasal secretions due to the depressed nasal bridge and everted or turned-out lips. Their teeth are either completely missing or, when present, they are crooked and sharp like vampire fangs.

People with this condition dont have sweat glands. Since they are unable to sweat, which dispels body heat, they are also unable to regulate their body temperature when exposed to the sun. Exposure to the sun or heat can make them incredibly warm. This uncontrollable body heat production (hyperpyrexia) can even be fatal.

Though we dont yet have a definitive cure for this condition, research is being done to find ways to inject the missing proteins into the amniotic sac between the 20th and 30th weeks of pregnancy, since sweat glands form at this time.

Hypohidrotic ectodermal dysplasia requires a multidisciplinary approach towards the management of symptoms:

For more information, please read our article onAlopecia: Causes, Diagnosis and Treatment.

Health articles in Firstpost are written by myUpchar.com, Indias first and biggest resource for verified medical information. At myUpchar, researchers and journalists work with doctors to bring you information on all things health.

Updated Date: Dec 18, 2019 21:38:20 IST

Tags : Allergy To Sun, Ectodermal Dysplasia, Fanged Teeth, Genetic Disease, NewsTracker, Rare Disease, Vampire Disease, X Chromosome

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This life-threatening disease has all the signs we've come to associate with vampires - Firstpost

Reviewed and Recommended by Erik Baquero