This life-threatening disease has all the signs we've come to associate with vampires
Sharp fangs, pale skin and allergy to sunlight - if you think we are talking about the vampires from Bram Stoker’s 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 we’ve come to associate with Dracula and his hoard, is called the vampire disease - medically known as Hypohidrotic ectodermal dysplasia.
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.)
What is vampire disease?
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.
Signs and symptoms
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 don’t 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.
Is the condition treatable?
Though we don’t 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:
- During hot weather, people with this condition must avoid physical exertion and have access to an adequate supply of water, cooling vests, air-conditioning, and a wet T-shirt to bring their body temperature down if required.
- To manage the dental issues, crowns, bridges, dentures and implants can be used according to the patient’s age and the thickness of their jaw bone. These prosthetics can help with functions like chewing and speaking to people.
- In people with alopecia or hair loss, customised wigs can be used.
- Nasal secretions need to be cleared off on a regular basis to help prevent or limit the severity of rhinitis (allergies).
For more information, please read our article on Alopecia: Causes, Diagnosis and Treatment.
Health articles in Firstpost are written by myUpchar.com, India’s first and biggest resource for verified medical information. At myUpchar, researchers and journalists work with doctors to bring you information on all things health.
The information provided here is intended to provide free education about certain medical conditions and certain possible treatment. It is not a substitute for examination, diagnosis, treatment, and medical care provided by a licensed and qualified health professional. If you believe you, your child or someone you know suffers from the conditions described herein, please see your health care provider immediately. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision. You acknowledge and agree that neither myUpchar nor firstpost is liable for any loss or damage which may be incurred by you as a result of the information provided here, or as a result of any reliance placed by you on the completeness, accuracy or existence of any information provided herein.
Updated Date: Dec 19, 2019 13:14:35 IST
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